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MetaCyc Pathway: superpathway of cholesterol biosynthesis

Pathway diagram: superpathway of cholesterol biosynthesis

If an enzyme name is shown in bold, there is experimental evidence for this enzymatic activity.

Superclasses: BiosynthesisFatty Acid and Lipid BiosynthesisSterol Biosynthesis

Some taxa known to possess this pathway include : Homo sapiens

Expected Taxonomic Range: Metazoa

The sequence of reactions in the cholesterol biosynthetic pathway may vary. This superpathway gives an overview of three possible routes of cholesterol biosynthesis. These routes exist because reduction of the carbon 24,25 double bond on the hydrocarbon side chain of the sterol ring structure by sterol Δ24-reductase can occur at multiple points in the pathway, giving rise to different intermediates. These intermediates, with or without a double bond in the hydrocarbon side chain, can serve as substrates for the other enzymes in the pathway. However, there is evidence that the preferred point of carbon 24,25 double bond reduction is directly after the isomerization of zymosterol [Bae97], as shown in pathway cholesterol biosynthesis I. In [Waterham01] and reviewed in [Gaylor02].

Subpathways: cholesterol biosynthesis I, cholesterol biosynthesis II (via 24,25-dihydrolanosterol), cholesterol biosynthesis III (via desmosterol), trans, trans-farnesyl diphosphate biosynthesis, mevalonate pathway I, lanosterol biosynthesis, epoxysqualene biosynthesis


Bae97: Bae SH, Paik YK (1997). "Cholesterol biosynthesis from lanosterol: development of a novel assay method and characterization of rat liver microsomal lanosterol delta 24-reductase." Biochem J 326 ( Pt 2);609-16. PMID: 9291139

Gaylor02: Gaylor JL (2002). "Membrane-bound enzymes of cholesterol synthesis from lanosterol." Biochem Biophys Res Commun 292(5);1139-46. PMID: 11969204

Waterham01: Waterham HR, Koster J, Romeijn GJ, Hennekam RC, Vreken P, Andersson HC, FitzPatrick DR, Kelley RI, Wanders RJ (2001). "Mutations in the 3beta-hydroxysterol Delta24-reductase gene cause desmosterolosis, an autosomal recessive disorder of cholesterol biosynthesis." Am J Hum Genet 69(4);685-94. PMID: 11519011

Other References Related to Enzymes, Genes, Subpathways, and Substrates of this Pathway

Abe94: Abe I, Tomesch JC, Wattanasin S, Prestwich GD (1994). "Inhibitors of squalene biosynthesis and metabolism." Nat Prod Rep 11(3);279-302. PMID: 15200015

Aharoni03: Aharoni A, Giri AP, Deuerlein S, Griepink F, de Kogel WJ, Verstappen FW, Verhoeven HA, Jongsma MA, Schwab W, Bouwmeester HJ (2003). "Terpenoid metabolism in wild-type and transgenic Arabidopsis plants." Plant Cell 15(12);2866-84. PMID: 14630967

Alber06: Alber BE, Spanheimer R, Ebenau-Jehle C, Fuchs G (2006). "Study of an alternate glyoxylate cycle for acetate assimilation by Rhodobacter sphaeroides." Mol Microbiol 61(2);297-309. PMID: 16856937

Aledo01: Aledo R, Zschocke J, Pie J, Mir C, Fiesel S, Mayatepek E, Hoffmann GF, Casals N, Hegardt FG (2001). "Genetic basis of mitochondrial HMG-CoA synthase deficiency." Hum Genet 109(1);19-23. PMID: 11479731

Altman71: Altman LJ, Kowerski RC, Rilling HC (1971). "Synthesis and conversion of presqualene alcohol to squalene." J. Amer. Chem. Soc. 1971 93:1782-3.

Anderson89: Anderson MS, Muehlbacher M, Street IP, Proffitt J, Poulter CD (1989). "Isopentenyl diphosphate:dimethylallyl diphosphate isomerase. An improved purification of the enzyme and isolation of the gene from Saccharomyces cerevisiae." J Biol Chem 1989;264(32);19169-75. PMID: 2681212

Anderson89a: Anderson MS, Yarger JG, Burck CL, Poulter CD (1989). "Farnesyl diphosphate synthetase. Molecular cloning, sequence, and expression of an essential gene from Saccharomyces cerevisiae." J Biol Chem 1989;264(32);19176-84. PMID: 2681213

Aoyama91: Aoyama Y, Yoshida Y (1991). "Different substrate specificities of lanosterol 14a-demethylase (P-45014DM) of Saccharomyces cerevisiae and rat liver for 24-methylene-24,25-dihydrolanosterol and 24,25-dihydrolanosterol." Biochem Biophys Res Commun 178(3);1064-71. PMID: 1872829

Aoyama94: Aoyama Y, Funae Y, Noshiro M, Horiuchi T, Yoshida Y (1994). "Occurrence of a P450 showing high homology to yeast lanosterol 14-demethylase (P450(14DM)) in the rat liver." Biochem Biophys Res Commun 201(3);1320-6. PMID: 8024575

Aoyama96: Aoyama Y, Noshiro M, Gotoh O, Imaoka S, Funae Y, Kurosawa N, Horiuchi T, Yoshida Y (1996). "Sterol 14-demethylase P450 (P45014DM*) is one of the most ancient and conserved P450 species." J Biochem (Tokyo) 119(5);926-33. PMID: 8797093

AVIGAN63: AVIGAN J, GOODMAN DS, STEINBERG D (1963). "Studies of cholesterol biosynthesis. IV. Reduction of lanosterol to 24,25-dihydrolanosterol by rat liver homogenates." J Biol Chem 238;1283-6. PMID: 13965665

Baker95a: Baker CH, Matsuda SP, Liu DR, Corey EJ (1995). "Molecular cloning of the human gene encoding lanosterol synthase from a liver cDNA library." Biochem Biophys Res Commun 213(1);154-60. PMID: 7639730

Balliano92: Balliano G, Viola F, Ceruti M, Cattel L (1992). "Characterization and partial purification of squalene-2,3-oxide cyclase from Saccharomyces cerevisiae." Arch Biochem Biophys 293(1);122-9. PMID: 1731628

Bard81: Bard M, Downing JF (1981). "Genetic and biochemical aspects of yeast sterol regulation involving 3-hydroxy-3-methylglutaryl coenzyme A reductase." J Gen Microbiol 1981;125(Pt 2);415-20. PMID: 7033470

Bard96: Bard M, Bruner DA, Pierson CA, Lees ND, Biermann B, Frye L, Koegel C, Barbuch R (1996). "Cloning and characterization of ERG25, the Saccharomyces cerevisiae gene encoding C-4 sterol methyl oxidase." Proc Natl Acad Sci U S A 93(1);186-90. PMID: 8552601

Barker82: Barker HA, Kahn JM, Hedrick L (1982). "Pathway of lysine degradation in Fusobacterium nucleatum." J Bacteriol 152(1);201-7. PMID: 6811551

Barnard81: Barnard GF, Popjak G (1981). "Human liver prenyltransferase and its characterization." Biochim Biophys Acta 661(1);87-99. PMID: 7295734

Bechtold72: Bechtold MM, Delwiche CV, Comal K, Gaylor JL (1972). "Investigation of the component reactions of oxidative sterol demethylation. Role of an endogenous microsomal source of reducing equivalents." J Biol Chem 247(23);7650-6. PMID: 4404598

Bennett84: Bennett MJ, Hosking GP, Smith MF, Gray RG, Middleton B (1984). "Biochemical investigations on a patient with a defect in cytosolic acetoacetyl-CoA thiolase, associated with mental retardation." J Inherit Metab Dis 7(3);125-8. PMID: 6150136

Benveniste02: Benveniste, P. (2002). "Sterol Metabolism." : March 27, 2002. The Arabidopsis Book. Rockville, MD: American Society of Plant Biologists. doi: 10.1199/tab.0004, http://www.aspb.org/publications/arabidopsis/.

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Please cite the following article in publications resulting from the use of MetaCyc: Caspi et al, Nucleic Acids Research 42:D459-D471 2014
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