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Increased biosynthesis of endoneurial oligohexosylceramides in human peripheral neuropathy

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Neurochemical Pathology

Abstract

Studying in vitro incorporation of [3H]galactose into biopsied sural endoneurium, we have demonstrated biosynthesis of glucocerebroside and its homologs, lactosylceramide, trihexosylceramide, and tetrahexosylceramide, in normal adult human sural nerve. Such in vitro biosynthesis of oligohexosylceramides is markedly increased in biopsied sural nerve from patients with various types of peripheral neuropathy. Since this altered glycosphingolipid biosynthesis occurs in several diversely caused polyneuropathies, it presumably relates to degenerative or regenerative events characteristic of neuropathy rather than a specific variety of neuropathy.

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Abbreviations

MLD:

Metachromatic leukodystrophy

DN:

Diabetic neuropathy

HMSN-III:

Hereditary motor and sensory neuropathy type III or Dejerine-Sottas disease

FA:

Friedreich’s ataxia

HSN-II:

Hereditary sensory neuropathy type II

HPTLC:

High-performance thin-layer chromatography

MGDG:

Monogalactosyl diacylglycerol

GlcCe:

Glucocerebroside

GalCe:

Galactocerebroside

GalCe-OH:

Galactohydroxycerebroside

Su:

Sulfatide

GL-1:

Monohexosylceramide

GL-2:

Lactosylceramide

GL-3:

Trihexosylceramide

GL-4:

Tetrahexosylceramide

PE:

Phosphatidylethanolamine

PI:

Phosphatidylinositol

PS:

Phosphatidylserine

PC:

Phosphatidylcholine

Sm:

Sphingomyelin

NL:

Nonpolar lipids

NGL:

Neutral glycosphingolipids

PNS:

Peripheral nervous system

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Authors and Affiliations

  1. Neurochemistry and Psychopharmacology Laboratory, Psychiatry Service, VA Medical Center (Highland Dr.), 15206, Pittsburgh, PA

    Jeffrey K. Yao & Peter James Dyck

  2. Peripheral Nerve Center, Mayo Clinic and Mayo Foundation, 55905, Rochester, MN

    Jeffrey K. Yao & Peter James Dyck

Authors
  1. Jeffrey K. Yao
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  2. Peter James Dyck
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Yao, J.K., Dyck, P.J. Increased biosynthesis of endoneurial oligohexosylceramides in human peripheral neuropathy. Neurochemical Pathology 7, 251–261 (1987). https://doi.org/10.1007/BF03160184

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  • DOI: https://doi.org/10.1007/BF03160184

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