Sphingolipids - Their metabolic pathways and the pathobiochemistry of neurodegenerative diseases

Abstract

Sphingolipids are building blocks of the plasma membrane of eukaryotic cells. Their function is to anchor lipid-bound carbohydrates to cell surfaces and to construct the epidermal water permeability barrier. Intermediates in sphingolipid metabolism are involved in the transduction of extracellular signals into the interior of cells. We give a review of the metabolism of these compounds in vertebrates, the intracellular topology of individual metabolic pathways, and the genetic diseases that occur as a result of defects in these pathways and which frequently lead to degeneration of the nervous system.