Cholesterol biosynthesis

Stable Identifier
R-HSA-191273
DOI
Type
Pathway
Species
Homo sapiens
ReviewStatus
5/5
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Cholesterol is synthesized de novo from acetyl CoA. The overall synthetic process is outlined in the attached illustration. Enzymes whose regulation plays a major role in determining the rate of cholesterol synthesis in the body are highlighted in red, and connections to other metabolic processes are indicated. The transformation of zymosterol into cholesterol can follow either of routes, one in which reduction of the double bond in the isooctyl side chain is the final step (cholesterol synthesis via desmosterol, also known as the Bloch pathway) and one in which this reduction is the first step (cholesterol biosynthesis via lathosterol, also known as the Kandutsch-Russell pathway). The former pathway is prominent in the liver and many other tissues while the latter is prominent in skin, where it may serve as the source of the 7-dehydrocholesterol that is the starting point for the synthesis of D vitamins. Defects in several of the enzymes involved in this process are associated with human disease and have provided useful insights into the regulatory roles of cholesterol and its synthetic intermediates in human development (Gaylor 2002; Herman 2003; Kandutsch & Russell 1960; Mitsche et al. 2015; Song et al. 2005).
Literature References
PubMed ID Title Journal Year
3524618 Regulation of cholesterol biosynthesis

Rudney, H, Sexton, RC

Annu Rev Nutr 1986
1390320 Cholesterol biosynthesis and metabolism

Russell, DW

Cardiovasc Drugs Ther 1992
12668600 Disorders of cholesterol biosynthesis: prototypic metabolic malformation syndromes

Herman, GE

Hum Mol Genet 2003
11969204 Membrane-bound enzymes of cholesterol synthesis from lanosterol

Gaylor, JL

Biochem Biophys Res Commun 2002
16054061 Insig-mediated degradation of HMG CoA reductase stimulated by lanosterol, an intermediate in the synthesis of cholesterol

Song, BL, DeBose-Boyd, RA, Javitt, NB

Cell Metab 2005
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