## Abstract A number of proteins are known to interact with huntingtin (htt), the Huntington's disease (HD) protein. Understanding the function of these htt‐associated proteins could help elucidate the pathogenesis of HD and the role that htt plays in the disease process. The present review focuses
Metabolic pathways and intracellular trafficking of gangliosides
✍ Scribed by Jose Luis Daniotti; Ramiro Iglesias-Bartolomé
- Publisher
- John Wiley and Sons
- Year
- 2011
- Tongue
- English
- Weight
- 319 KB
- Volume
- 63
- Category
- Article
- ISSN
- 1521-6543
- DOI
- 10.1002/iub.477
No coin nor oath required. For personal study only.
✦ Synopsis
Abstract
Gangliosides constitute a large and heterogeneous family of acidic glycosphingolipids that contain one or more sialic acid residues and are expressed in nearly all vertebrate cells. Their de novo synthesis starts at the endoplasmic reticulum and is continued by a combination of glycosyltransferase activities at the Golgi complex, followed by vesicular delivery to the plasma membrane. At the cell surface, gangliosides participate in a variety of physiological as well as pathological processes. The cloning of genes for most of the glycosyltransferases responsible for ganglioside biosynthesis has produced a better understanding of the cellular and molecular basis of the ganglioside metabolism. In addition, the ability to delete groups of glycosphingolipid structures in mice has been enormously important in determining their physiological roles. Recently, a number of enzymes for ganglioside anabolism and catabolism have been shown to be associated with the plasma membrane, which might contribute to modulate local glycolipid composition, and consequently, the cell function. © 2011 IUBMB IUBMB Life, 63(7): 513‐520, 2011
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