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Topic: Cryptobiotic soil vs. good images (Read 9178 times)
Sr. Member
Posts: 679
Cryptobiotic soil vs. good images
Reply #20 on:
October 13, 2004, 11:08:48 PM »
[font color=\'#000000\']
There is a place in Death Valley near the Dunes where you can still see wagon tracks that are 100 to 150 years old. The place heals slowly. Perhaps you think one person cannot do a lot of damage, but multiply that by, say, a hundred thousand. You will not be the only one who wants to get a bit closer. Once a path is started, more people will follow. There are places where the John Muir Trail is worn hip deep with several new parallel paths.
Howard, the earth can most likely support many more people than currently live on it today. But there is a large difference between simply living and living what most of us believe to be a quality life.
Here in the west our milk and meat producers have discovered that they can increase the number of livestock that they raise by cramming them into feed lots. Now, do you want to take photographs of cattle spread across a rangeland or photographs of cattle crammed into pens? Do you want to live the equivalent life of a cow restricted to a small space, standing in your own filth?
Please go back and read the posts in this thread and I think you will see that some of the complaints that people have made are largely due to the stresses that our population size have made on our natural resources. We are having to restrict ourselves to fixed paths in order to not trample too much of a slow healing landscape.
Now to both Howard and "Sfleming".... Let me ask you both a favor.
Please consider ceasing to engage in the divisive behavior that is so tearing our culture apart. Name calling and characterizing groups of people by extreme examples does no good in the attempt to solve problems. Please make an effort to listen to
sides, communicate your ideas and concerns in an objective and helpful manner. We, as a country and a world, would be much better off if we quit treating issues as we would a sports engagement. You know, I win. You, therefore must loose.[/font]
Sr. Member
Posts: 339
Cryptobiotic soil vs. good images
Reply #21 on:
October 14, 2004, 12:34:07 AM »
[font color=\'#000000\']That's fine Bob but I don't feel like being polite when extremist environmentalists are working hand in glove with eletist leftist academics and most of the main stream media to revolutionize American society. My society. I for one am not going quietly.
I don't care to live in a socialist Big Brother world and it is plain that the Left is radically undermining western society. Environmentalism has become the spearpoint of the assault. It has to be not just resisted but fought because the end result of a command and control economy run by an oligarchy of academics and political scientists will be indeed a Brave New World.
Human freedom is what this country is all about. Yes it does not work without a responsible population and sadly many many of us have lost or not developed the intestinal fortitude to accept and shoulder necessary personal responsibility. The answer is not to imprison people within a police state run by eletes. Rather it is to educate and require people to exhibit character.
I have a quote hanging on my wall by John Adams: "We have no government armed with power capable of contending with human passions unbridled by morality and religion. Avarice, ambition, revenge or gallantry would break the strongest cords of our Constitution as a whale goes through a net. Or constitution was made only for a moral and religious people. It is wholly inadequate to the government of any other."
The natural state of man is to be inslaved by kings and despots. That is the history of us. This country has the oldest constitutional republic on earth and most other western constitutions were modeled on ours. We all grew up in, by all history's example, a very strange situation. We are free. I'm not willing to surrender my freedom to a bunch of scare mongers shrieking 'global warming' or any other such poppycock.
Peolpe who try and tell me and others that Giant Sequois are still being harvested need to be resisted strongly. I'm sorry if you don't like my tone. I don't like your intentions.[/font]
howard smith
Sr. Member
Posts: 1237
Cryptobiotic soil vs. good images
Reply #22 on:
October 14, 2004, 09:32:25 AM »
[font color=\'#000000\']Bob, before you check out, please tell me where I was name calling. I reread this and other similar threads and just didn't see it. Any name calling was unintended.
Your intention to lead a good life and leave the earth a better place is admirable. There is a huge difference between leading "a good life" and living "the good life." The only group I have been critical of are those living "the good life" who now want to save it all and deny "the good life" to others. Those are the folks I see as being opposed to any kind of develoment because it ruin their enjoyment (the good life) of the environment. I personally don't think they really care all that much about the snail darter or spotted owl. Those creatures are simply the rally cry. "Save the spotted owl" sounds much better than "Save my playground."
I have referred to them as "Hollywooders." I see them as the people who have the good life and can now afford to pay three times what steak is worth. So they want to get rid of ugly feed lots in thename of the cows. They don't want to live in poluted air, so they drive electric cars powered by electricity made in someone else's backyard where someone else get to breate the poluted air.
I would add to that group those who oppose development in Iceland. They say it will ruin the pristine wilderness. That Icelanders don't need it. After all, they have a per capita GNP that is the envy of much of the world. Maybe so. But I also see on this site from a MR workshop attendee in that pristine wilderness that everything cost three times what i was worth. Maybe Icelanders need all that GNP if they want lettuce and tomato on that cod fish sandwich. Why should I have that simply because I have three times what its worth?
There are a lot of people on our planet. As the population grows, the space available to grow food decreases while the demand for food increases. Hence, a more practicle reason for feed lots than corporate greed.
No Bob, I don't want to live like a cow crammed into a feed lot. Nor do I want to live "the good life" of a range cow, free to roam about before the truck ride to McDonald's. But thus is the life of a cow. But I think that is what cows are for.[/font]
Sr. Member
Posts: 679
Cryptobiotic soil vs. good images
Reply #23 on:
October 14, 2004, 12:04:57 PM »
[font color=\'#000000\']Howard, I'm sorry that you thought I was including you in the "please, no name calling" request. If you read that into my post, my bad.
I will repeat my request that we not try to make our points by using extreme examples, often very non-representing examples, of those who have a different opinion.
If I wanted to make a pro-environmental statement I could bring up the logger who recently drained his Cat oil into a local creek when changing his oil, the local guy who intentionally cut down several trees containing nesting egrets, the person who took a chain saw and girdled one of the largest redwoods in the area.
But those people are not representative of the loggers in our area. Most of the loggers and timber workers are good, decent people. And they are truly troubled by the behavior of these extreme, out of control individuals, just as the vast majority of environmentalists are disgusted by such activities as burning SUVs and damaging logging equipment.
We've gone from a country where there was plenty of room for people to do as they pleased. But the days when we could trash an area, move on, and let the environment recover are over. We've gone from a few wagons moving over fragile terrain to millions of feet and tires. That means that we have conflicts between people who want to make a living by harvesting/extracting natural resources and people who want to enjoy nature.
If we allow ourselves to get caught up in name calling and other divisive behavior we are going to spend a lot more time getting to some reasonable solutions. And we spend a lot of time with our knickers in knots rather than enjoying life.
Me? I prefer to be free range for as long as I've got until I head for the charcoal grill. (Actually, I'm getting interested in some of the new 'non-embalmed, non-concrete' burials rather than cremation. I'd rather be slowly taken up by a tree than adding to the global warming problem. )
howard smith
Sr. Member
Posts: 1237
Cryptobiotic soil vs. good images
Reply #24 on:
October 14, 2004, 04:06:55 PM »
[font color=\'#000000\']AGW, it is my understanding that National Parks here in the US are to preserve unique areas of the US for people now and in the future to enjoy. I think they do a good job of that. Especially considering the high uses some areas get.
Forty years ago, I would drive to Yosemite Valley an any Friday night and find plenty of available camp sites. But now, well, first-come-first-serve is just not an option. To get a camp site this Friday night would nean you would have to arrive two weeks ago and wait. I can live with hard to get reservations. Or go somewhere else. There are planty of palces where you can still just arrive. Just not Yosemite Valley in July.
I hiked across the Grand Canyon (South-North and back) a few years ago. It was difficult to get one of the few permits to camp below the rim. I had to camp two extra nights on the norther rim to make it work. While talking with the Park Service, I learned that on Memorial Day (first summer holiday in the US) before the quota system ws put in place, there were nearly 3000 campers at the Colorado River campground at Phantom Ranch. When I visited, it was crouded with a few hundred. It is easy to see why some areas need extra protection.
On the other hand, there are plenty of places where few people go and access is pretty free and easy. The reason is so few people go there, there is no need to limit the visits. Yet. I really do feel limited access to certain areas is for the good of the area and the visitors, today and next year. The rules aren't there just to keep you from getting that great photo.
I like to go to North Coyotte Buttes in Utah/Arizona. There is a limit of 10 people per day. I go in August because that when I can go, and it is relatively easy to get one of the 10 permits 6 months in advance. Mainly because it is HOT. I have been there many times and most often I see no one else, or a single group of 2 or 3 people. Frankly, I prefer this to "SRO" crouds.
Some rules are in place for the least common denominator visitor. For instance, I have been in Death Valley many times. Stop at the visitor's center and ask about going to the Race Track. "4WD only. Very hard drive." I've been there in a Volvo wagon and a VW van. But the 70 year old tourists in their rented car from Las Vegas airport could get into serious trouble. So, the blanket advice is to not go, rather than, it is OK for you but not for them. Especially when they may be more up for the trip than you are. The Park Service doesn't want any one to have a bad time or get rescued, or worse.
Bob, I did read it that way. Sorry. And thanks for helping me. My reference to Hollywooders is meant to address people of wealth and high visibility. Some think we should all drive electric cars. I know I can't afford it now. I can't understand why I should vote for John Kerry because Cher thinks Connie Rice is an idiot. I saw a TV item with the proud actress showing off her organic garden in Malibu. We should all have one andlive like she does - so aware of the environment. Then she waters here tomatoes with water from the Feather River brought to LA through the California Aquaduct. If you have eaten lettuce in the US, it was probably grown in the Southwestern desert - totally unfit to grow much of anything but lizards and cactus but for water from the Colorado River, saved behind the Glenn Canyon, Hoover and other dams.
Bob, I am a retired nuclear engineer. I had a 35 year career, mostly in nuclear power generation. I think I understand what environmental extremists , well meaning or other wise, can do. And I also understand the risks of technology.[/font]
Sr. Member
Posts: 679
Cryptobiotic soil vs. good images
Reply #25 on:
October 14, 2004, 05:26:29 PM »
[font color=\'#000000\']Howard, I said that I was going to withdraw from this discussion. I guess I lied. ;o)
I'm a retired psychologist. I can assure you that there are plenty of crazy people on both sides of every issue. (And even some crazy people who haven't taken sides. ;o)
To pick out one, or a few, of the most extreme on an issue to support your disagreement with that position does rational discussion no favors. I think we would be much, much better off if we refrained from the 'talk show' behavior of belittling the ideas of others and tried to understand their concerns and to clearly communicate ours.
I'm so tired of politics as a competitive sport....[/font]
Ron Pfister
Posts: 4
Cryptobiotic soil vs. good images
Reply #26 on:
October 14, 2004, 06:32:04 PM »
[font color=\'#000000\']Interesting debate. What troubles me in all this is that most of you talk about how we, human kind, will be affected by our own land use strategies as far as our recreational behavior is concerned. There's much more to it than that, IMO. ANY open space that's preserved is good. Our fauna and flora suffered and still suffers tremendously by our most often unsustainable land use practices.
Case in point: I just returned from a trip to the Extremadura-region in western Spain, where agricultural practices are still relatively traditional and diverse, and there is lots of open space. What a huge difference in bird diversity compared to Switzerland, where I live. Large-scale monocultures and urban sprawl have robbed many bird species of their habitat here. Their numbers have declined, as they can't move elsewhere. There is no elsewhere!
The park management strategy that works best in my view is what I experienced in the national parks of Botswana (southern Africa): High price, very poor infrastructure = low visitor volume. Not a single tarred road (in fact, gut-wrenching road conditions most of the way). You pay more than USD 100 admittance for a vehicle and two persons per day. Camp sites consist of a mowed spot under a tree with a little number plate on it. Toilet facilities are monopolized by Baboons ;-). You're not allowed to leave your vehicle either (except at camps) - but considering the fact that there may be a pride of lions hiding in the tall grass a few meters away from you, you may not want to, anyway...
I agree, this is an elitist approach to conservation if you beleive that everyone is entitled to the recreational benefits that nature reserves can provide us with in the short term. But the fauna and flora are surely the merrier, and that's what's far more important in the long run IMO.
Posts: 9
Cryptobiotic soil vs. good images
Reply #27 on:
October 14, 2004, 07:02:18 PM »
[font color=\'#000000\']Thanks for the Link Howard....There is a very useful page which sets out the rationale behind the selection of National Parks and their operation.
I'm assuming that those who are complaining are doing so in relation to park policy rather than to the concept of the National Park itself. I would therefore recommend that they follow the link and get a better understanding about what the park service are trying to achieve.
It is also clear from the ancillary info on the site that there is whole structured hierachical approach to natural heritage conservation in the US. From NP's, through State facilities to local designations and places managed by NGO's. There are also huge tracts of land which are unaffected by designation or restriction.There are recreational areas and there are conservation areas etc....in actual fact it seems prety well sorted.
I'm now facinated by crypyobiotic soils and I'm off to find out more about them.
Peter McLennan
Sr. Member
Posts: 1799
Cryptobiotic soil vs. good images
Reply #28 on:
October 07, 2004, 11:49:03 AM »
[font color=\'#000000\']I've come across this issue many times in my travels in the desert southwest and I've come to the opinion that "Leave nothing but footprints" is a bad idea. When I'm walking in the wilderness I pretend that I'm a secret agent on a covert mission and leaving footprints is the LAST thing I want to do.
It's usually pretty easy to avoid the cryptobiotic soil, once you know what it looks like. There are plenty of rock outcroppings, gravel beds, etc. that you can use to get yourself to your shooting location. Walking on rock or gravel leaves not a trace. A few seconds thought and a minor detour can eliminate all traces of your passage.
It's easy, all it takes is a little conscious thought.[/font]
howard smith
Sr. Member
Posts: 1237
Cryptobiotic soil vs. good images
Reply #29 on:
October 07, 2004, 04:00:59 PM »
[font color=\'#000000\']Yet another layer of government is not the solution. People can be clean and considerate if they want to be. Spanking the bad ones doen't seem cost effective to me. Finger printing the tossed can would only work if the culprit had his prints on file. Not all of do. It would cost plenty, and in the end, a shrewed lawyer would say the finger prints only proved the culprit had touched the bottle, not thrown it away.
Many states have a deposit on bottles and cans. If you return the item, you get your deposit back. If you throw it away, it is still worth a nickel for someone to pick it up. Make the deposit big enough, and no one would throw it away. Bigger yet, and you might get mugged for your cans.
Some places have trail head limits on the number of people that can enter an area at any given time. This allows for a better wilderness experience and reduces impact on some fragile areas. Some areas are closed to camping altogether.
I have been to a few parks in Canada and New Zealand. The first thing I noticed was how pristine clean they were. So, people can take care of their parks if they want to.
It has also been my experience that the farther from the parking lot you get, the less trash and heavy impact you see. Yes, part of the reason is fewer people go there, but also the more serious the hikers, the better care they seem to take of parks and public places.[/font]
howard smith
Sr. Member
Posts: 1237
Cryptobiotic soil vs. good images
Reply #30 on:
October 09, 2004, 08:05:52 PM »
[font color=\'#000000\']I know places in Yosemite where you can spend several hours alone that are less than a half mile from the roads. It can be done. There are no trails to these places but no signs that say don't go.
I saw on the news today that a visitor to Yellowstone was scalded when he left the trail and broke through a thicn crust into very hot water. Another reason to follow the rules.[/font]
Sr. Member
Posts: 339
Cryptobiotic soil vs. good images
Reply #31 on:
October 12, 2004, 11:16:56 PM »
[font color=\'#000000\']I would never advocate letting people's houses burn, but fire is a part of the natural process. One of the reasons fire is a problem now is because we have attempted to control the natural process for too long. Undergrowth and dead wood have been allowed to pile up while we do everything we can to stop fires. Nature can take care of itself, but we insist on meddling.
I especially don't like elitist morons who wear funny looking pointy hats acting like god's own wardens running roughshod over my high-tax-paying rights.
I'm assuming you're not living in the US, because if you are, you are not paying high taxes. I'm not sure that paying taxes gives anyone the right to destroy something to obtain a good photograph.
I believe the welfare of the subject should always come first. I have no idea what crypto-biotic soil looks like, and I have no intention of leaving the trail in case I walk on it.[/font]
[font color=\'#000000\']Spoken like a true believer who has never bothered to search and study OUTSIDE his own faith.
Yes fire is natural but naturally fire would sweep through forested lands in intervals of decades ... not centuries. Because we followed Smokie's precepts for so long forests became cluttered. The smart thing to do would be to log them, clear the brush and replant. The invirowhako thing to do is to keep out the loggers for no good reason and let them burn. Thus precluding logging for another century. Why do they hate logging so much? Because they love Salmon? Salmon can be protected through logging operations done intelligently. When thousands of acres of forest overgrown for a century burn ... everything dies and it takes a long time for it to come back. So why do the tree-huggers advocate such foolish practices? I think it is because they are anti-human-anti-capitalist and thus anti-freedom.
You also make the slanderous assertion that I advocate destroying something because I pay taxes. I think I made it pretty clear that I personally am very aware of the wilderness and my environment and that I believe in nurturing both. Your statement simply reveals your prejudice and dishonesty.
As to taxes: Just what is 'high' to your mind. Right now ... all told ... I surrender about 50% of my income .... most of which goes to support lazy irresponsible people who can not manage to make their way in life. You want more?
And back to the wonderful 'crypto' ... 'biotic'. Don't you wonder about that nomenclature? I certainly do. I think it's bullshit.
If you read my other posts in this thread you will see that I advocate more money for support of our wildlands and I advocate that those who use them do the payin. I also advocate licensing and educating to protect them. What's your plan?[/font]
Sr. Member
Posts: 339
Cryptobiotic soil vs. good images
Reply #32 on:
October 13, 2004, 03:17:55 AM »
[font color=\'#000000\']I have literally no respect for environmentalists any longer. They've been caught in far too many lies and deceits. Whether it's planting lynx fur in Minnesota, reintroducing wolves where they are not needed, protecting cougars where they kill children and eat women hikers or preserving crocs in Austraila where they can eat more people.
The environmentalists are extreme. Yet they alway accuse industrialists of crimes against nature. I'd like to see a happy medium. Thousands of lives were impacted and the price of lumber has been doubled by the Spotted Owl scam. This was an out and out lie.
Same thing is being done right now in Ca. with the red legged frog. Plant one frog in one drainage ditch and a billion dollar development can be killed.
Of course thats just fine because 'development' is evil. Development means jobs and houses for people to live in.
Here in Central Texas the environmentalists have gotten near total control of development using the lever of the Edwards Aquifer. This aquifer MUST be kept nearly full all year round. Why? Because of an indroduced non indigenous species of minnow and a salmander. These live in non natural springs that were dynamited out of the hills in the thirties and have been netted out prior to severe droughts in the past when the springs did dry up ( the aquifer was still 90% full) and then were later reintroduced where they flourished.
Of late a 4 golf course and several hundred home development was quashed after years of planning by these 'nature lovers'.
So ... yes I call environmentalists names. I think they are charlatans with a hidden agenda. I don't have to wonder about this. I know it. The Left went underground and came back up calling themselves environmentalists. Their goals have remained unchanged. To institute a command and control economy ruled by an elete few.[/font]
howard smith
Sr. Member
Posts: 1237
Cryptobiotic soil vs. good images
Reply #33 on:
October 13, 2004, 09:20:30 AM »
[font color=\'#000000\']People are a natural part of the earth's environment, no matter how you think we got here (except perhaps by spaceship from somewhere else). I see the problem to be that people are the only species on earth that is capable of making huge changes to the environment. So it may be a matter of degree. A beaver builds a am. Cool. Humans build a dam. Bad. The beaver pond eventually fills with dirt and a meadow is born. Cool. Lake Meade fills with dirt. Bad. Both change the world, ony to a different degree. Some environmentalists say the beaver dam is natural, Hoover Dam is man-made. True, but not the whole truth. Humans are natural so what they do is natural so Hoover Dam is natural. Humans use concrete, not mud and trees.
A small beaver dam benefits a few beavers. Hoover Dam benefits millions of people.[/font]
Sr. Member
Posts: 339
Cryptobiotic soil vs. good images
Reply #34 on:
October 13, 2004, 01:03:04 PM »
[font color=\'#000000\']Excellent points Howard.
I would like to try and inject a droplet of truth into Bob's seemingly closed mind.
NO Giant Sequoias are threatened with logging. Not for many decades. There shall never be a Giant Sequoia harvested ... EVER again. There is limited logging of the Dawn and Coastal species of Redwood which are much lesser species which grow rather quickly compared to their altitude loving bigger cousins.
This is how environmentalists spread their propaganda and misinformation. This sort of manipulation of the public mind is their stock and trade. You can't make any headway with them either because we are talking religion here. Mother Earth is their goddess and they will do anything to protect her. Spreading lies is justified because the cause is so righteous.
In the beginning the environmentalists did humankind a great favor. Back then they were called conservationist and I think that was a more honest word. We WERE acting in a near oblivious manner to our surroundings and horrible crimes were committed against the land we all must live on and from. Most of the wrongs have been wholly stopped in the US. The rest of the world will catch up when the big picture economics of it all hits home. This is now happening in China. The industrialists ( all sons and daughters of the old guard millitary) are realizing that piles of money are not so fun if you and your children cannot breathe the air.
But now the environmentalists carry on as if no progress has been made. They cry ever louder and trumpet their alarmist BS ever more hysterically. They come at us preaching fire and brimstone and ever lasting damnation if we do not reverse the industrial age. It's just nonsense.
Human population and science got us into this situation and that is the only way to proceed. We must continue to develop non poluting tech and teach responsibility to our kids. Population pressure is lessening as the entire world becomes industrialized. Europe and America only grow due to immigration now. Sensible progress can still be made. The screeching of the treehugging alarmists needs to stop. They are actually have a reverse effect to their goals now. The sky is not falling and there is no wolf. There are concerns and dangers. They will be faced and dealt with.
Perhaps we shall cause some global warming. This will not harm the planet. Some folks may have to relocate. Some folks will get a better climate. Some will get worse. It will happen so gradually that the population moves will not even be realized by the affectees at the time.
We WILL come up with an alternative to fossil fuels long before it could become the problem the treehuggers say it is right now. Or we won't and we will go back to the stone age. None of us will see it.
I still maintain that the environmentalists (the leaders) have a hidden agenda and it is political.[/font]
Sr. Member
Posts: 679
Cryptobiotic soil vs. good images
Reply #35 on:
October 14, 2004, 01:17:31 AM »
[font color=\'#000000\']My intentions are to lead a good life and to try to leave the earth in a little better condition than how I found it. I'm sorry you don't like my intentions.
(Let me clear up a little misconception/understanding. The redwoods in my neck of the woods are coastals, not Sequoias. We've already cut so many of the Sequoias that we keep the few remaining in a couple of inland parks.)
"Sfleming", you sound like a very angry person. I'm going to drop out of this discussion (it has little to do with photography anyway). I hope you find a way to relax a bit and try to see issues from more than one extreme position. The world is a very complex place....[/font]
howard smith
Sr. Member
Posts: 1237
Cryptobiotic soil vs. good images
Reply #36 on:
October 14, 2004, 04:09:34 PM »
[font color=\'#000000\']AGW, I got so whippd up, I forgot. Try
howard smith
Sr. Member
Posts: 1237
Cryptobiotic soil vs. good images
Reply #37 on:
October 14, 2004, 06:48:07 PM »
[font color=\'#000000\']Elitist? Yep. Pretty soon only Hollywooders will be able to fly to Africa and ebjoy nature. Pay the $100 or whatever, jump into their Landrover and go see the wilds. I'm happy for them. Maybe I will able to see their National Geogrphic special.[/font]
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text: Employment Current Openings from the Office of Career Services are listed below Posted: 3/6/2012Time Warner Cable/ OSS Software Engineer/ Syracuse, NY FCC Unit_TWC: HQ450 Controlling Establishment ID: 00247 - Syracuse Court POSITION TITLE: OSS Systems Engineer Job Requisition Number: 134086BR Application WebSite: http://jobs.timewarnercable.com/job/Syracuse-OSS-Systems-Engineer-Job-NY-13201/1760898/? feedId=40&campaignId=3&utm_source=maximus&utm_campaign=J2W_Maximus Details: Posting Job Title: OSS Systems Engineer Requisition #: 134086BR Posting Location: United States - New York - Syracuse Area of Interest: Engineering/Technical Operations Position Type: Full Time Posting Job Description: PURPOSE: The Systems Engineer for system deployment and support efforts of regional applications. This support provides access to element management and development environments that support the development organization as well as the engineering organizations with performance and health of service delivery platforms. ESSENTIAL DUTIES AND RESPONSIBILITIES: Provides direct support of the TWC Network Management Systems including Netcool (Impact, Reporter, Webtop, Visionary), JDSU's NetAnalyst & NetOptimize suite of products, Cisco Transport Manager (CTM), Alcatel 5620 SAM, Lucent INC, and others. Participates in a team of engineers responsible for development and support of numerous Regional OSS applications supporting Residential and Commercial services for Time Warner Cable. Participates in the team developing the support infrastructure, including metrics development, process improvement, and the OSS tool enhancement life cycle. Assists the management with evaluations of the current OSS tools suite and recommendation for enhancements as well as next generation solutions. The successful candidate will be responsible for support of all commercial off the shelf (COTS) products and developing/support JOB QUALIFICATIONS: Provide exceptional end user support of all OSS tools for the Northeast/National Region. Assist management defining enhancements needed to the existing OSS platform in order to meet current and future business needs. Participate with cross-functional groups within TWC to define requirements for OSS support improvements. Work with vendors to assist with the installation and support of OSS applications. Assist management defining and implementing support metrics and SLA's. Provide technical leadership and solutions to management. Provide technical trouble shooting support as needed. Assist management developing tactical and strategic application support roadmap Development of a technical support knowledge base Provide 24x7 on call support as necessary Strong Unix skills, including Solaris and Linux operating systems. Must be able to write scripts using shell and Perl. Database administration experience with MS SQL Server, Oracle and MySQL. Strong experience with Network Management tools is essential. Excellent PC Software skills in Microsoft Excel, Word, and PowerPoint as well as experience with reporting tools such as Crystal Reports. Strong troubleshooting and analytical skills measured by the ability to solve complex technical issues Excellent written and oral communication skills are required. EDUCATION/EXPERIENCE REQUIRED: Bachelor's degree (BS/BA) from a four year-college or university or equivalent experience and education. Current Cisco Certified Network Associate (CCNA) or equivalent certification is preferred Minimum of 5 years network and systems work experience. Experience preferred with troubleshooting industry-standard Network and Systems equipment, including Ethernet, SONET, DWDM and High-Speed Data Cable equipment (CMTSs). Familiarity with Netcool, Hobbit, Big Brother, Remedy as well as platform-based tools such as Cisco Transport Manager, Alcatel SAM is a plus. TRAVEL REQUIREMENTS AND FREQUENCY: Travel within the East Region Business Job No. 1884

subset: commoncrawl


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CORRECTION: An earlier version of this story gave the wrong state for Rep. Tom Cotton. He is from Arkansas.
President Obama gave the last press conference of his first term on Monday, setting the stage for a new term that has the potential to look a lot like the old one. Just as it was after the 2010 midterm elections, the president finds himself negotiating with a Republican-held House with a large batch of fresh faces and various crises on the horizon. As I noted in an earlier article, a full 38 percent of the House has served for less than three years, the highest percentage since the 1994 Gingrich revolution.
That means there's plenty of room for newbies to stand out, and there's no one way to do that. Here are four different freshmen taking four different paths to stand out as leaders:
- The Experienced One: It seems like an oxymoron for someone in the freshman class to have "experience." By very definition they are new to the job. But Rep. Richard Hudson, R-N.C., isn't new to the halls of the Capitol. He has served as chief of staff for three members of Congress, so he knows the inside and out of the lower chamber. This could allow Hudson to be both a trusted newbie and a sage wiseman at the same time. "I can't stand up on a desk and tell them all to follow me," he said on his first day in Congress earlier this month. "But we need to come to a consensus about what our objectives are, and who our leader is going to be. The lesson we've learned these last couple of years is that we need to agree as conservatives and then fight for what we believe."
- The Young Gun: There aren't a lot of guys who managed to be liked by both the outside tea-party groups and the leadership in the House of Representatives. But Rep. Tom Cotton, R-Ark., has managed to do just that. The 35-year-old retired Army veteran may have just been elected to the House, but his name has already been floated as candidates to run against Democratic Sen. Mark Pryor in 2014.
- The Outsider: If you don't know who Rep. Ted Yoho is, then you probably haven't been paying much attention to the House these past few weeks. The former large-animal veterinarian made a splash with his first vote on his first day in office when he cast his ballot for Rep. Eric Cantor to be speaker of the House. This is a guy who says he doesn't owe his leadership anything, and that having spent years dealing with large deadly animals isn't about to be intimidated by people in his party.
- The Operative: As reported by my colleague Shane Goldmacher, before Ann Wagner had even won her seat as a representative from Missouri, she had already introduced herself to 34 members of her new class and had contributed money and campaigned for other GOPers around the country. It's the kind of political move that comes from someone who knows how to play the game, and having served as a cochair of the Republican National Committee from 2001 to 2005, Wagner knows how to do just that. "She just has so much potential and skill sets," House Majority Whip Kevin McCarthy, R-Calif., told National Journal Daily. "I expect big things from Ann all around."


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The packable Mrs. Davis straw floppy hat
While poolside fashion usually brings to mind tropical swim trunks and everything "itsy bitsy", the truth is that the pool warrants just as much of a fashion statement as any other setting. Featuring 1940's inspired fashion with a modern twist, we styled our Poolside collection of packable summer hats with fresh pool outfits from Mystery Mister. From retro one-pieces, Don Draper-esque swim shorts, to chic cover-ups, our friends from Mystery Mister, a great little vintage shop in the famous Haight and Ashbury neighborhood of San Francisco, was chock-full of amazing clothes to pair with our hats. See the highlights from our Poolside photo shoot, and make sure to check out Mystery Mister the next time you're in the city by the Bay!


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You are hereHome › Programs & Events › Readings & Conversations ›
Poems By Heart: The Art and Joy of Memorization with Carol Conroy
Memorization Circle
Date and Time:
December 8, 1998 - 7:00PM to 9:00PM
Event Location:
Poets House, Spring Street
Poems By Heart brings people together to recite from memory the poems they love. Participants are asked to memorize a new poem each month that someone else has written.
Event Sponsored By:
Poets House
Event Type:
Readings and Conversations
Upcoming Readings and Conversations
Current Exhibition
June 12, 2015 to September 19, 2015


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Annual Holiday Dinner, December 2, 2012
The Friends of Ithaca College rang in the holidays at their 2012 annual dinner and business meeting. President Tom Rochon spoke to the Friends about the importance of their support and briefed the group on some of the latest accomplishments at the college. Outgoing Friends president George Conneman then passed the baton to incoming president Art Ostrander. The musical portion of the evening included performances by the Ithaca College Marimba Band as well as our wonderful musical theatre students. When the IC Jazz Ensemble hit the stage, the Friends headed onto the dance floor, and the evening concluded with dancing and much holiday merriment.


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Summer Hand-tied
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Just like a summer garden in full bloom, this pretty hand-tied bouquet is bursting with colour, texture and variety. For this selection we included lots of favourites including charming sweet Williams, classic dahlias and a timeless pink rose as the centrepiece.
Featuring pink dahlias, cream spray chrysanthemums, cerise rose, green carnations, purple September flowers, mixed sweet williams and green alchemilla mollis hand-tied with salal and presented with gift wrap finished with a lime ribbon.
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A collection of essays by Jean-Paul Sartre that touch upon the subject of existentialism by looking at aesthetics, emotions, writing, phenomenology, and perception The Philosophy of Existentialism collects representative essays on Jean-Paul Sartre's pioneering subject: existentialism. Beginning with a thoughtful introduction by fellow French philosopher Jean Wahl, this work looks at existentialism through several lenses, exploring topics such as the emotions, imagination, nothingness, freedom, responsibility, and the desire to be God.
By providing exposition on a variety of subjects, The Philosophy of Existentialism is a valuable introduction to Sartre's ideas.
©1965 Philosophical Library (P)2013 Audible, Inc.
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National Coffee Drinking Trends 2013
Order Your Copy Today!
NCA's National Coffee Drinking Trends (NCDT) has been the industry's gold standard for over 60 years, consistently tracking annual coffee consumption trends of U.S. consumers since 1950.
Whether you're a marketer, roaster, manufacturer, trader, wholesaler or distributor, NCDT 2013 is the vital resource to help you better target your best customers, strategically market your brand and plan for future products.
As in past editions, NCDT 2013 provides a broad range of key data and analysis about American coffee drinkers – average daily consumption for the past day, week and year; how, where and when coffee is consumed, including both home and workplace consumption; how often coffee is consumed, during which times of day, and in what quantities; consumption by life-stage and age, and much, much more.
You'll also find assessments of consumer attitudes about various coffee drinks, flavor additives, and the effect of the economy on U.S. coffee consumption.
Here is a few highlights from this year's study:
Coffee Consumption Jumps by 5%, 83% of Americans Say They Drink Coffee on a Past Year Basis
Hispanic-Americans consume Espresso-based Beverages at twice the rate of other groups on a Past Day Basis
- Single-cup segment builds on growth, ownership of single-cup brewers increases to 12%
With so much valuable data available in the 2013 edition of National Coffee Drinking Trends, you owe it to yourself and your business to order your copy today. Why wait?
Order the National Coffee Drinking Trends 2013 today and you'll recieve your electronic copy today* and the print copy within a week.
Order Your Copy Today!
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Data tables, Individualized Analysis Report and Custom Data also available. Please call 212-766-4007 for more information.
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smart lux bassinet
A luxury deep bassinet for your smart lux stroller.
Not currently available in this region.
smart lux bassinet is HOT! because:
- convert your stroller seat frame into a bassinet
- clicks easily onto your stroller frame
- freestanding for use off the stroller
- carry using the bumper bar from your stroller frame
- natural bamboo fabric interior
- plush mattress base & padded sides
- 2 piece fitted bamboo sheet set for bassinet mattress (sold separately)
- durable & wipe clean 600d exterior fabric
- removeable top cover (lid)
- deep & spacious for a growing baby
- bassinet sun cover and storm cover available (sold separately)
Your smart lux frame cleverly converts to become the bassinet frame. This bassinet includes the following parts:


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Guest blog post from Nancy Perry, Senior Vice President of ASPCA Government Relations.
Californians, your hard work has paid off! Yesterday, Governor Jerry Brown signed the state's bill to ban hounding (S.B. 1221) into law. The new law bans the practice of releasing radio-collared dogs loose in the woods to chase and tree bears and bobcats, all so trophy "hunters" can shoot the terrified animals down from point-blank range. California now joins the more than 30 states that do not permit this blatant and needless form of animal cruelty.
We started work early on this legislation, partnering with the many groups and shelters that attended our 2012 California Humane Lobby Day. Hundreds of advocates flooded the Capitol, where we held a rally for the hound dogs and wildlife—and of course, hound hunters showed up to oppose us and the ban. They were determined to preserve this unsporting pastime despite ample evidence of the grotesque abuses inherent to hounding.
Even under such pressure, humane voices won the day. Every time we asked for your help, you responded in full force. Your work has changed your state forever and made it a safer haven for thousands of animals. If you live in California, please take a moment to thank Governor Brown.
In addition to the hounding ban, Governor Brown signed two other great bills for companion animals:
Declawing/devocalization (S.B. 1229): Landlords are now expressly prohibited from discouraging potential tenants from applying for housing if their pets are not declawed or devocalized.
Cost of care in animal abuse cases (S.B. 1500): This measure clarifies existing law so that anyone accused of animal abuse must provide for the cost of care for animals seized from them. This helps shelters tremendously, and also helps ensure that animals will not end up back in the hands of their abusers.
As a California native, I was thrilled to join our California advocates in-person and work on this legislation! But I'm excited to announce that we now have Sacramento-based Kevin O'Neill, our new Western Region State Legislative Director, aboard to guide us as we take on new challenges in California, Oregon, Washington and Nevada. Welcome, Kevin—may this be just the beginning of great things from your states!
Want help us change laws to protect animals? Join the ASPCA Advocacy Brigade!
From the bottom of our hearts, we want to thank you. A few weeks ago, we told you about the hay emergency facing American horse caregivers this year. We told you that persistent drought has put hay in dangerously short supply, and that if we don't pull together and do something, horses will die.
We asked you to help us feed horses by contributing to our Hay Bale-Out initiative, and we were overwhelmed by the generosity of those who answered our call.
Here's what happened next.
We told equine rescue groups the good news: The ASPCA has your back, and we can provide hay to help get your horses through the winter. We asked them to tell us what they needed.
Since then, equine groups have been flooding us with requests.
We're deep in the process of working closely with these groups to meet their hay needs, and our equine rescue friends tell us this assistance couldn't come at a better time.
"This year is setting up to become a 'perfect storm' for equine neglect, as we are already seeing a huge increase in calls and skinny horses," Gail Vacca of the Illinois Equine Humane Center told us. "Thank goodness many will find relief due to the efforts and support of the ASPCA."
We're so grateful for your help feeding horses this year. If you haven't yet given to this critical effort, it's not too late! Every little bit counts, and it all goes directly to feeding horses.
Watch ASPCA.org for updates on this life-saving project, and if you haven't already, consider being a part of it.
Ever wondered if microchips are worth it? If you ask Amanda L., they sure are! Here's her story:
We adopted our cat Dempsey in January 2007 from the Houston SPCA. Dempsey is the smartest cat we've ever met. He plays fetch, drinks water from the faucet and follows you everywhere.
So we were heartbroken when, shortly after a moving to Ann Arbor, Michigan, in 2010, we lost Dempsey. We put up fliers, stayed up at night listening for him and searched the neighborhood for weeks, but with no luck.
We moved to New York City the following year but never stopped thinking about Dempsey and wondering where he could be and whether he was even alive.
Then, one Saturday while we were sitting in the park reading, we received an email from a vet in Ann Arbor—they found Dempsey!
An amazing woman had noticed him around her yard all summer and had been feeding him and looking out for him. They had warmed to each other, and so she decided to take him to a vet to see if he belonged to anyone. The first thing the vet did was scan him and they found our information. It was almost exactly two years to the day since we had lost Dempsey. We still cannot believe it!
Dempsey is currently recuperating with his grandparents in Michigan, and we are going to see him soon. We owe our reunion with Dempsey entirely to the kindness of a stranger and his microchip. We are so thankful that we have him back in our lives!
For more info on why microchips are awesome, check out this video.
When Britney came to the ASPCA through the Humane Law Enforcement department, she was in pain and suffering from multiple untreated conditions.
Fortunately, the veterinary staff at the ASPCA's Bergh Memorial Animal Hospital was able to nurse Britney back to health, and eventually she became available for adoption; we could finally find a home for this sweet dog who had suffered so much.
We knew it would be a challenge.
Statistically speaking, animals who are black, large or senior, as well as those who take medication or can't live with other animals, usually spend more time waiting for homes. Britney was all of these things, and to make matters worse, she didn't engage much with adopters who passed her habitat, preferring to rest quietly.
Still, we knew that the right person would come for Britney, and to speed things along, we spread the word about this special canine. After seeing Britney's picture and reading about her, Kevin Bechard tells us, "I wanted to snatch her right up."
A few days later Bechard was going above and beyond to help Britney settle in to her new home in suburban Connecticut.
"The first couple nights I actually slept on the floor with her because she can't do stairs, and she would just reach out with her paw and make sure it was against me," he recalls. "Only then she would allow herself to go to sleep. If I moved away a little bit, her eyes would open."
The love and comfort of a forever home has revived Britney, who has blossomed into a trusting and happy pup: Bechard reports that at his house Britney loves romping outdoors and goes on multiple nature walks a day.
In return, Bechard has gained a new best friend who, he says, never required house-training or manners instruction, thanks to her age. "I wish more people would consider older dogs," Bechard says. "I was open to anything, but I was so pleasantly surprised."
As the crisp fall air settles in and bright orange and red leaves swirl down from the trees, what better time to hit the trail? But as you plan your fall wilderness hikes, don't forget your canine friends! Here are some ASPCA expert tips to ensure your dog's safety as you explore the great outdoors together.
- Pack a leash. With so many nooks and crannies to explore on the trail, it's best to opt for a non-extending leash to avoid potential tangles with branches and brambles.
- Bring IDs, please! Always make sure that your current contact information, including your cell phone number, is attached to your dog's collar or harness.
- Check your records. You never know what you might encounter on a hike through the woods. Before you set out on your journey, make sure your pet's vaccinations are up to date.
- Give pests the boot. Tick prevention is essential when tromping through the great outdoors. Treat your pooch with PetArmor, a spot-on flea and tick treatment by FidoPharm, one of the ASPCA's corporate partners.
- Leave no trace. Scoop up after your dog when she goes to the bathroom as you would on a stroll through your neighborhood.
- Stay hydrated—don't forget to bring enough water for yourself and your dog. It's best to avoid letting your dog drink from nature's water fountains, as puddles, lakes and streams can be home to nasty parasites and toxins that could be harmful to your furry friends.
For more tips about keeping your pet safe in the wilderness, check out our expert hiking safety tips, and feel free to download our printable Hiking Safety Tips guide [PDF] to carry with you on the trail.


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The Community of Disc Golfers and About All Things Disc Golf
just got mine :) so nice
Views: 353
Had mine for a little over a year now. The best bag I've owned.
That is a great looking bag and interesting set up. I DO really like the pocket for a jacket, towel, etc.
I have this bag:
I use it with Innova Back Straps. I like having the two bottle holders. I use one for a bottle and the other for my two towels, so they don't hit the ground and pick up spurs or whatever.
And, I love the flexy putter pocket and being able to carry 22 discs. But, carrying 35 would be cool, too. Also, I wish the pockets were bigger on my bag. And the top mesh pocket is useless when it is rolled up...although I have found it can hold gum and mini chocolates and still be rolled up...good for when I need a little sugar boost on the course.
Had mine about a year now and it's holding up well. I set it down in the grass, concrete, dirt, mud, etc. No excessive wear that I can see. I really like the putter pocket on the end, makes it handy to grab the putter and make the putt without taking off the bag when using backstraps. That large front pocket is also insulated and can hold many beverages if need be. I had as many as 30 discs in my bag, but it was just too heavy to carry all day so I backed it down to 16 plus 2 putters. The bag can be configured to suit anyone's needs. It took me a while to get the bag to hang just right with my backpack straps, but I finally got it. It is a heavy bag even when empty, but that adds to the durability. It did come with a special 4 hook-up mono-strap, but because the bag is so hefty, I recommend the quad-shocks.
I suspect this bag will last me many, many years and I play eveyday. A good quality bag for sure.
ive had my bag for almost 2 years now. this is the best bag i have owned and the only problem im having right now with it is the umbrella loops wearing down cause the velcor is coming unstitched.. other than that i have no complaintswith this bag..
Anyone have a grip eq bag? They seem to always be sold out. :(
Look now. your welcome:)
They have some on their web site for sale, but you better hurry!
Very nice bag for sure. Here is an added touch to make it hold up longer. I bought the Innova "U" channel soft frame and slipped it in the main compartment. Fits just right and it adds another layer of protection from your discs moving around and scuffing up the inside of the bag. You can get them at discgolfvalues for around $9.00
Thx for the heads up guys... I ended up buying one online last night. Can't wait till it arrives. :)
You'll dig it. It a well designed bag. I also have the Mahal and although it's built much better than the Grip. I think the Grip blows it away in the design dept. Heck I got fed up with trying to set up the Mahal (Yoke and straps). So I just put it awat until my patience returns :)
© 2015 Created by Terry "the Pirate" Calhoun.


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snug carrycot sunhood
Add a splash of color to your snug ™ carrycot.
Not currently available in this region.
snug carrycot sun hood is HOT! because:
- add a splash of color to your snug carrycot !
- golden kiwi, sky and cherry; a perfect match with the legacy navigator stroller
- chilli, apple and flint: a perfect match with the DOT stroller
- comes easily on and off your snug carrycot


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The Penn Museum accepts donations of documented ethnographic and archaeological materials that are free of major conservation concerns for the express purpose of preservation, research, exhibition, and/or public education. Objects from antiquity, if exported from their country of origin after 1970, will be considered only if the donor can provide evidence documenting their legal exportation in accordance with the Museum's 1970 Pennsylvania Declaration and the 1970 UNESCO Convention on the Means of Prohibiting and Preventing the Illicit Import, Export, and Transfer of Ownership of Cultural Property. Additionally, the Museum adheres to pertinent U.S. laws and international treaties regarding cultural properties.
If you would like to donate objects to the Penn Museum, please send all inquiries to the Senior Registrar (see contact info below) and include the following:
All donation proposals are first reviewed by the Museum's relevant Curatorial Sections and then further reviewed by the Museum's Acquisitions Committee. This Committee meets twice a year (typically May and December) and makes recommendations to the Museum Director for final approval. For donors wishing to have their donation considered in time for a year-end charitable contribution, inquiries with all required information should be submitted no later than November 20 of the relevant year. The Museum has an extended holiday vacation each December and will process last-minute donations early in the following year unless special arrangements are made in advance.
A few other key items to note:
(215) 898-7900
3260 South Street
Philadelphia, PA 19104
(215) 898-4000
Tuesday-Sunday: 10:00am - 5:00pm
First Wednesdays: 10:00am - 8:00pm
Monday: CLOSED


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This story was updated at 4:05 p.m. Dec. 4, 2012.
Residents of the Whetstone Apartments in Gaithersburg describe the area as �really quiet,� safe, and pleasant. But the quiet row of apartments was the scene of a fatal stabbing early Monday.
Bruce Goldfarb, a special assistant to the state�s Chief Medical Examiner said Tuesday that Cesar Gregory Smith, 45, of Gaithersburg, died from multiple stab wounds to the torso. Smith was found dead in a unit of the apartment complex at 22 Whetstone Drive, Montgomery County police said.
Shortly after midnight, officers from the Gaithersburg Police Department and Montgomery County police responded to a report of a shooting at the Whetstone Apartments at 22 Whetstone Drive in Gaithersburg, according to Montgomery County police.
Police found Smith in an apartment unit, having suffered �blunt force trauma� to his body, Montgomery County police spokesman Capt. Paul Starks said.
Smith was pronounced dead on the scene by county Fire and Rescue personnel, according to police.
Starks said police had not yet confirmed the nature of the association between Smith and his killers and that the investigation is still ongoing.
Smith was in the apartment with another man when multiple suspects entered the apartment and separated the two, police said. After a brief amount of time, the suspects were heard arguing with the victim and then left the apartment, police said.
The surviving man found the injured victim inside the apartment and called police.
Investigators believe that the crime was not random and that the victim and suspects may have known each other, Starks said.
A resident who lives in a building nearby said she heard shots.
�I just heard a pow, pow, pow,� she said.
Clifton Bowie, a neighbor who has lived in the complex for the last five years, said such incidents are unheard of.
�I haven�t heard of even any break-ins,� he said.
�[Smith] was a loner and didn�t fool with too many people,� said Delah Jay, a woman living in a nearby apartment.
He was dating a friend of hers, she said.
�I was up until 1:30 a.m. and I didn�t hear anything,� said Sharee Henson, who lives in an apartment building across the lot from where police found Smith.
Henson said Smith, or �Goldie,� was a friend of her mother�s.
�This is really scary,� she said, adding that she walks by the apartment on her way to her job at a nearby mall.
�That doesn�t sit well with me,� she said.
A manager of the apartment complex said Smith was not a resident. Starks said that the apartment was one of the former places where he had lived, and that he may have lived in more than one place.
Police are asking anyone with information regarding this crime to contact the Major Crimes Division at 240-773-5070. Those who wish to remain anonymous may call Crime Solvers of Montgomery County at 866-411-TIPS (8477). Crime Solvers will pay a cash reward of up to $1,000 for information that leads to an arrest and/or indictment for this crime.
Staff Writer Kara Rose contributed to this report.


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Interpretive Handbook
89494 :
Tripeptidyl Peptidase 1 (TPP1) and Palmitoyl-Protein Thioesterase 1 (PPT1), Leukocytes
Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
The neuronal ceroid lipofuscinoses (NCL) comprise a group of recessively inherited neurodegenerative disorders involved in lysosomal protein catabolism. Clinically they are characterized by vision loss, seizures, mental regression, behavioral changes, movement disorders, and the accumulation of storage material with a characteristic appearance by electron microscopy. Currently, at least 10 genetically distinct NCLs (CLN1-CLN10) are known. The age of onset and rate of deterioration varies according to type of NCL. Tissue damage is selective for the nervous system and many patients die in the first decade of life due to central nervous system degeneration. There is an overall incidence in the United States estimated at 1 in 12,500.
Children affected by infantile NCL (CLN1) typically have normal growth and development until about 6 to 12 months of age. Slowed head growth occurs at around 9 months followed by psychomotor degeneration, seizures, and progressive macular degeneration leading to blindness by the age of 2. CLN1 is caused by a deficiency of the lysosomal enzyme palmitoyl-protein thioesterase 1 (PPT1), which cleaves long-chain fatty acids (usually palmitate) from cysteine residues. Electron microscopy shows granular osmophilic deposits (GRODs) in most cell types. PPT1 is thought to play an active role in various cell processes including apoptosis, endocytosis, and lipid metabolism. Infantile NCL has an incidence of 1 in 20,000 in Finland and is rare elsewhere.
The late infantile form of NCL (CLN2) is caused by deficiency of the lysosomal enzyme tripeptidyl peptidase 1 (TPP1), which cleaves tripeptides from the N-terminus of polypeptides. Tissue damage results from the defective degradation and consequent accumulation of storage material with a curvilinear profile by electron microscopy. There is widespread loss of neuronal tissue especially in the cerebellum and hippocampal region. Disease onset occurs at 2 to 4 years of age with seizures, ataxia, myoclonus, psychomotor retardation, vision loss and speech impairment.
Diagnostic strategy depends on the age of onset of symptoms. In children presenting between the ages 0 to 4 years, enzyme assay of PPT1 and TPP1 is an appropriate first step. In addition, molecular genetic testing of CLN1 or CLN2 may allow for identification of the disease causing mutations.
Useful For Suggests clinical disorders or settings where the test may be helpful
Evaluation of patients with clinical presentations suggestive of neuronal ceroid lipofuscinoses (NCL)
An aid in the differential diagnosis of infantile and late infantile NCL
Interpretation Provides information to assist in interpretation of the test results
Tripeptidyl peptidase 1 (TPP1) and palmitoyl-protein thioesterase 1 (PPT1) enzyme activity below 5 nmol/hour/mg of protein are highly suggestive of late-infantile and infantile neuronal ceroid lipofuscinoses, respectively.
Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
This assay does not detect carrier status of neuronal ceroid lipofuscinoses.
Some variants with an age of onset occurring in older individuals have been noted.
Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
TPP1: 85-326 nmol/hour/mg protein
PPT1: 20-93 nmol/hour/mg protein
Clinical References Provides recommendations for further in-depth reading of a clinical nature
1. Haltia M: The neuronal ceroid-lipofuscinoses: from past to present. Biochim Biophys Acta 2006;1762:850-856
2. Kavianen R: Juvenile-onset neuronal ceroid lipofuscinosis with infantile CLN1 mutation and palmitoyl-protein thioesterase deficiency. Eur J Neur 2007;14:369-372
3. Enns GM, Steiner RD, Cowan TM: Lysosomal disorders. In Pediatric Endocrinology and Inborn Errors of Metabolism. Edited by K Sarafoglou, GF Hoffmann, KS Roth, New York, McGraw-Hill Medical Division, 2009, pp 749-750


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Portsmouth Museum of Art
One Harbour Place
Portsmouth, NH 03801
Ph: 603-436-0332
The Portsmouth Museum of Art is a community-centered museum presenting the work of emerging and established artists - local, national and international. They are a contemporary art museum dedicated to showing artwork of the 21st century from national and international emerging artists.
They offer exhibitions and programs that educate the public about art, as well as to use art as a tool to generate conversation about current events, other cultures, social issues and a wide range of other topics with a diversity of viewpoints. Their goal is to enrich the local cultural landscape by developing partnerships, creating collaborative opportunities among artist and art organizations an by presenting a complete art experience that includes literature and poetry, performance art, film and other technologies and to initiate an on-going public dialogue around art.
Parking is available between Bow and State Streets directly across from the museum. Guided tours are available by appointment.
Website: www.portsmouthmfa.org
Email: firstname.lastname@example.com
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