Lipid abnormalities in hereditary neuropathy. Part 4. Endoneurial and liver lipids of HMSN-III (Déjerine-Sottas disease)

Jeffrey K. Yao, Peter J Dyck

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Abstract

Detailed lipid analyses of 1 liver and 4 endoneurial samples obtained from patients with hereditary motor and sensory neuropathy, Type III (Déjerine-Sottas disease) were compared with controls. In parallel with the severe decrease in myelin, there was a substantial alteration of lipid composition including: (1) a decrease in endoneurial content of free cholesterol, glycosphingolipid and phospholipid; (2) a selective reduction of endoneurial phosphatidylserine; (3) a high proportion of 18:0 in endoneurial triacylglycerol and phospholipids; and (4) a striking diminution of long chain fatty acids (C22-C27) in sphingolipids. No abnormal profile of liver glycosphingolipids was found. Contrary to what we reported previously, these studies do not confirm a systemic glycosphingolipid abnormality in this disorder.

Original languageEnglish (US)
Pages (from-to)179-190
Number of pages12
JournalJournal of the Neurological Sciences
Volume52
Issue number2-3
DOIs
StatePublished - 1981

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Hereditary Sensory and Motor Neuropathy
Glycosphingolipids
Lipids
Liver
Phospholipids
Sphingolipids
Phosphatidylserines
Myelin Sheath
Triglycerides
Fatty Acids
Cholesterol
Lipid III

ASJC Scopus subject areas

  • Aging
  • Clinical Neurology
  • Surgery
  • Neuroscience(all)
  • Developmental Neuroscience
  • Neurology

Cite this

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title = "Lipid abnormalities in hereditary neuropathy. Part 4. Endoneurial and liver lipids of HMSN-III (D{\'e}jerine-Sottas disease)",
abstract = "Detailed lipid analyses of 1 liver and 4 endoneurial samples obtained from patients with hereditary motor and sensory neuropathy, Type III (D{\'e}jerine-Sottas disease) were compared with controls. In parallel with the severe decrease in myelin, there was a substantial alteration of lipid composition including: (1) a decrease in endoneurial content of free cholesterol, glycosphingolipid and phospholipid; (2) a selective reduction of endoneurial phosphatidylserine; (3) a high proportion of 18:0 in endoneurial triacylglycerol and phospholipids; and (4) a striking diminution of long chain fatty acids (C22-C27) in sphingolipids. No abnormal profile of liver glycosphingolipids was found. Contrary to what we reported previously, these studies do not confirm a systemic glycosphingolipid abnormality in this disorder.",
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AU - Dyck, Peter J

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N2 - Detailed lipid analyses of 1 liver and 4 endoneurial samples obtained from patients with hereditary motor and sensory neuropathy, Type III (Déjerine-Sottas disease) were compared with controls. In parallel with the severe decrease in myelin, there was a substantial alteration of lipid composition including: (1) a decrease in endoneurial content of free cholesterol, glycosphingolipid and phospholipid; (2) a selective reduction of endoneurial phosphatidylserine; (3) a high proportion of 18:0 in endoneurial triacylglycerol and phospholipids; and (4) a striking diminution of long chain fatty acids (C22-C27) in sphingolipids. No abnormal profile of liver glycosphingolipids was found. Contrary to what we reported previously, these studies do not confirm a systemic glycosphingolipid abnormality in this disorder.

AB - Detailed lipid analyses of 1 liver and 4 endoneurial samples obtained from patients with hereditary motor and sensory neuropathy, Type III (Déjerine-Sottas disease) were compared with controls. In parallel with the severe decrease in myelin, there was a substantial alteration of lipid composition including: (1) a decrease in endoneurial content of free cholesterol, glycosphingolipid and phospholipid; (2) a selective reduction of endoneurial phosphatidylserine; (3) a high proportion of 18:0 in endoneurial triacylglycerol and phospholipids; and (4) a striking diminution of long chain fatty acids (C22-C27) in sphingolipids. No abnormal profile of liver glycosphingolipids was found. Contrary to what we reported previously, these studies do not confirm a systemic glycosphingolipid abnormality in this disorder.

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