Central nervous system: cholesterol turnover, brain development and neurodegeneration
- 23 January 2009
- journal article
- review article
- Published by Walter de Gruyter GmbH in Biological Chemistry
- Vol. 390 (4), 287-293
- https://doi.org/10.1515/bc.2009.035
Abstract
The average amount of cholesterol in the whole animal equals approximately 2100 mg/kg body weight, and 15% and 23% of this sterol in the mouse and human, respectively, is found in the central nervous system. There is no detectable uptake across the blood-brain barrier of cholesterol carried in lipoproteins in the plasma, even in the newborn. However, high rates of de novo cholesterol synthesis in the glia and neurons provide the sterol necessary for early brain development. Once a stable brain size is achieved in the adult, cholesterol synthesis continues, albeit at a much lower rate, and this synthesis is just balanced by the excretion of an equal amount of sterol, either as 24(S)-hydroxycholesterol or, presumably, as cholesterol itself.Keywords
This publication has 55 references indexed in Scilit:
- Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1 −/− mouseProceedings of the National Academy of Sciences of the United States of America, 2009
- GM2/GD2 and GM3 gangliosides have no effect on cellular cholesterol pools or turnover in normal or NPC1 miceJournal of Lipid Research, 2008
- The Cholesterol Absorption Inhibitor Ezetimibe Acts by Blocking the Sterol-Induced Internalization of NPC1L1Cell Metabolism, 2008
- Feedback regulation of cholesterol synthesis: sterol-accelerated ubiquitination and degradation of HMG CoA reductaseCell Research, 2008
- NPC1L1 (Niemann–Pick C1-like 1) mediates sterol-specific unidirectional transport of non-esterified cholesterol in McArdle-RH7777 hepatoma cellsBiochemical Journal, 2007
- Receptor-mediated and bulk-phase endocytosis cause macrophage and cholesterol accumulation in Niemann-Pick C diseaseJournal of Lipid Research, 2007
- Cholesterol substrate pools and steroid hormone levels are normal in the face of mutational inactivation of NPC1 proteinJournal of Lipid Research, 2006
- Protein Sensors for Membrane SterolsCell, 2006
- Niemann-Pick C1 Like 1 Protein Is Critical for Intestinal Cholesterol AbsorptionScience, 2004
- Increase in neutral cholesteryl ester hydrolase activity produced by extralysosomal hydrolysis of high-density lipoprotein cholesteryl esters in rat hepatoma cells (H-35)Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1994