This book gives a comprehensive and critical discussion of important developments in studies concerning the above subject. This text discusses the structure, function, and metabolism of glycosphingolipids, which are related to the study of sphingolipid storage diseases. Inborn defects of metabolism are found in Gaucher's and Fabry's disease, which are characterized by lipid accumulation in the brain. Another paper reviews the chemical and genetics of critically lysosomal hydrolase deficiencies that can cause the storage of sphingolipids.
This book then explains the role of myelin basic protein in lipids in vivo that the weak bonding of the protein is not a major component of myelin stability. Another paper discusses the procedures for isolating subfractions of myelin and myelin-related membranes, with some attention given on the alterations in the subfractionation of myelin in pathological hypomyelinating and demyelinating conditions. Another article discusses the biochemical and enzymatic composition of lysosomes and the biosynthesis, intracellular transport, storage, and the degradation of lysosomal constituents.
This collection of papers will benefit scientists doing research in microbiology, microchemistry, molecular genetics, and neurochemistry.
- ISBN: 9781483153599 |
- Hardcover: 634 pages |
- Publisher: Elsevier Science |
- Publication date: Oct 22, 2013 |
- Language: English |
- Format: Ebook