Increased biosynthesis of endoneurial oligohexosylceramides in human peripheral neuropathy.

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.