Premium
The Biochemistry of Sphingolipid Storage Diseases
Author(s) -
Sandhoff Konrad
Publication year - 1977
Publication title -
angewandte chemie international edition in english
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.831
H-Index - 550
eISSN - 1521-3773
pISSN - 0570-0833
DOI - 10.1002/anie.197702733
Subject(s) - sphingolipid , phosphorylcholine , enzyme , ceramide , moiety , biochemistry , membrane , chemistry , sphingomyelin , biology , microbiology and biotechnology , stereochemistry , apoptosis
Sphingolipids are components of animal membranes that are made up of a hydrophobic ceramide and a hydrophilic moiety (phosphorylcholine, oligosaccharides or their derivatives). Several enzymes are required for the step‐by‐step degradation of the sphingolipids. If one of these enzymes is deficient or totally lacking, then its substrates accumulate in the body and cause severe damage, especially when storage occurs in the nervous system. The enzyme defects are inherited in a recessive manner.