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Lipid abnormalities in hereditary neuropathy part 3 plasma and erythrocyte lipids of hereditary motor and sensory neuropathy type iii dejerine sottas disease



Lipid abnormalities in hereditary neuropathy part 3 plasma and erythrocyte lipids of hereditary motor and sensory neuropathy type iii dejerine sottas disease



Journal of the Neurological Sciences 38(1): 59-66



Plasma glucosylceramide levels of 5 patients with Hereditary Motor and Sensory Neuropathy Type III (Dejerine-Sottas disease) were approximately 50% higher than in controls. Erythrocyte glucosylceramides were within the range of normal values. An abnormality of lipid metabolism may underlie this recessively inherited hypertrophic neuropathy. No abnormality was observed among other glycosphingolipid fractions. An altered sphingomyelin fatty acid composition and reduced phosphatidylcholine level was also found, but this may not be disease-specific.

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Accession: 005814285

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