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[Abnormalities of N-linked oligosaccharide moieties of glycoproteins in patients with myotonic dystrophy].

Myotonic dystrophy (MyD) is an inheritant disease which results from an abnormal expansion of trinucleotide repeat of myotonin protein kinase. MyD has been considered as one of membrane diseases as several functional disturbances were detected on the plasma membranes in patients with MyD. We reported here that some proteins and tissues in patients with MyD took only incomplete processing of N-glycans. The characteristic feature of the oligosaccharide moieties was so unique that N-glycans of glycoproteins such as immunoglobulin G, proteins on the erythrocyte membrane and the insulin receptor revealed reduced content of N-acetylneuraminic acid and galactose. In other words, these glycoproteins in patients with MyD had N-linked oligosaccharides which terminated with N-acetyglucosamine at the non-reducing ends. These specific defect of glycosylation may indicate the disturbances of the sarcoplasmic reticulum or the Golgi apparatus and be one of the factors which construct the membrane disorder observed in patients with MyD.