{'fraction_non_alphanumeric': 0.023779724655819776, 'fraction_numerical': 0.01877346683354193, 'mean_word_length': 5.837606837606837, 'pattern_counts': {'":': 0, '<': 0, '<?xml version=': 0, '>': 1, 'https://': 0, 'lorem ipsum': 0, 'www.': 0, 'xml': 0}, 'pii_count': 0, 'substrings_counts': 0, 'word_list_counts': {'cursed_substrings.json': 0, 'profanity_word_list.json': 0, 'sexual_word_list.json': 0, 'zh_pornsignals.json': 0}}
Phrenic and Hypoglossal motor unit pathology in a Spinocerebellar ataxia type 7 mouse model

Spinocerebellar ataxia type 7 (SCA7) is an autosomal dominant neurodegenerative disorder caused by a deleterious CAG repeat expansion in the coding region of the ataxin‐7 gene on chromosome 3. Patients with infantile SCA‐7 have the largest repeat expansion (>200 repeats) and the most severe disease characterized by progressive loss of coordination, dysarthria, dysphagia and retinal degeneration. Death in these infants results from severe hypotonia, aspiration pneumonia and respiratory failure. To understand the mechanism behind aspiration and respiratory failure in SCA7, we comprehensively examined hypoglossal (XII) and phrenic neuropathology of a mouse model of infantile SCA7: the SCA7‐266Q mice.