Central nervous system: cholesterol turnover, brain development and neurodegeneration
TLDR
High rates of de novo cholesterol synthesis in the glia and neurons provide the sterol necessary for early brain development in the mouse and human, respectively.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.read more
Citations
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Malformation syndromes caused by disorders of cholesterol synthesis
Forbes D. Porter,Gail E. Herman +1 more
TL;DR: In this paper, the authors reviewed clinical and basic science aspects of Smith-Lemli-Opitz syndrome, desmosterolosis, lathosterolosis and HEM dysplasia.
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Cholesterol metabolism in neurons and astrocytes
Frank W. Pfrieger,Nicole Ungerer +1 more
TL;DR: An understanding of cholesterol metabolism in the brain and its role in disease requires a close look at individual cell types, including neurons and astrocytes, which are identified as net producers and consumers of cholesterol.
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Liver X receptors in lipid signalling and membrane homeostasis.
Bo Wang,Peter Tontonoz +1 more
TL;DR: The role of LXRs in cholesterol and lipid metabolism and the emerging strategies to target the LXR pathway to treat metabolic diseases are discussed.
Thematic Review Series: Genetics of Human Lipid Diseases Malformation syndromes caused by disorders of cholesterol synthesis
TL;DR: Clinical and basic science aspects of Smith-Lemli-Opitz syndrome, desmosterolosis, lathosterolosis), HEM dysplasia, X-linked dominant chondrodysplasia punctata, Congenital Hemidyspl Asia with Ichthyosiform erythroderma and Limb Defects Syndrome, sterol-C-4 methyloxidase-like deficiency, and Antley-Bixler syndrome are reviewed.
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The Role of Cholesterol in the Pathogenesis of NASH
TL;DR: This review examines the effects of excess FC in hepatocytes, Kupffer cells (KCs), and hepatic stellate cells (HSCs), and the subcellular mechanisms by which excess FC can induce cellular toxicity or proinflammatory and profibrotic effects in these cells.
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