Cholesterol Biosynthesis Pathway (Homo sapiens)

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Fatty Acid DegradationLanosterinSQLENSDHLCYP51A1SC4MOLFDFT1Mevalonic acid-5PDHCR7SqualeneMVKHMG-CoASC5DLIDI1Mevalonic acidGeranyl-PPisopentenyl pyrophosphateMevalonic acid 5-pyrophosphateFDPSCholesterolHMGCS1Acetyl-CoALathosterol7-DehydrocholesterolPMVKMVDHMGCRfarnesyl pyrophosphateDimethylallylpyrophosphateLSS(S)-2,3-EpoxysqualeneFDPS


No description


GenMAPP notes 
References: Bard, M., Bruner, D. A., Pierson, C. A., Lees, N. D., Biermann, B., Frye, L., Koegel, C., and 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, 186-190.

Gaylor, J. L. (2002). Membrane-bound enzymes of cholesterol synthesis from lanosterol. Biochem Biophys Res Commun 292, 1139-1146.
Li, L., and Kaplan, J. (1996). Characterization of yeast methyl sterol oxidase (ERG25) and identification of a human homologue. J Biol Chem 271, 16927-16933.
Liu, X. Y., Dangel, A. W., Kelley, R. I., Zhao, W., Denny, P., Botcherby, M., Cattanach, B., Peters, J., Hunsicker, P. R., Mallon, A. M., et al. (1999). The gene mutated in bare patches and striated mice encodes a novel 3beta-hydroxysteroid dehydrogenase. Nat Genet 22, 182-187.
Ohashi, M., Mizushima, N., Kabeya, Y., and Yoshimori, T. (2003). Localization of mammalian NAD(P)H steroid dehydrogenase-like protein on lipid droplets. J Biol Chem 278, 36819-36829.
Sagami, H., Korenaga, T., and Ogura, K. (1993). Geranylgeranyl diphosphate synthase catalyzing the single condensation between isopentenyl diphosphate and farnesyl diphosphate. J Biochem (Tokyo) 114, 118-121.
Yoshida, Y., Noshiro, M., Aoyama, Y., Kawamoto, T., Horiuchi, T., and Gotoh, O. (1997). Structural and evolutionary studies on sterol 14-demethylase P450 (CYP51), the most conserved P450 monooxygenase: II. Evolutionary analysis of protein and gene structures. J Biochem (Tokyo) 122, 1122-1128.

GenMAPP remarks 
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Ontology Terms



  1. Porter FD; ''Malformation syndromes due to inborn errors of cholesterol synthesis.''; J Clin Invest, 2002 PubMed Europe PMC Scholia


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106703view13:12, 17 September 2019MaintBotHMDB identifier normalization
106355view21:38, 21 August 2019KhanspersModified description
105554view06:13, 9 August 2019KhanspersModified description
105182view12:46, 17 July 2019DeSlOntology Term : 'classic metabolic pathway' added !
102317view07:56, 20 December 2018FehrhartChanged title due to overlap with Reactome pathway
101819view10:05, 10 November 2018EgonwReplaced a wrong CAS number of with right ChEBI identifier.
98746view21:23, 3 October 2018Khanspersupdated pathway node
91292view19:13, 23 January 2017KhanspersQuick edit to datanode annotation or property
90349view01:29, 6 November 2016AlexanderPicofixed width on metabolites
90348view01:25, 6 November 2016AlexanderPicoFixed pathway node and conversion interactions
90347view01:12, 6 November 2016AlexanderPicoupdated group type
90344view01:00, 6 November 2016AlexanderPicofixed IPP reactions
81059view14:15, 19 July 2015EgonwAdded a reference for the cholestorol synthesis track.
78758view12:57, 23 January 2015Bart SmeetsPeriodical save, work in progress
78708view01:36, 17 January 2015AndtheWingsModified title
78107view14:04, 3 December 2014Bart Smeetsremoved SREBF1/2, these regulate nearly all enzymes in this pathway.. better to leave them out entirely than to make it seem that HMGCS1 and HMGCR are their only targets
78106view13:48, 3 December 2014Bart Smeetschanged all catalytic reactions to the appropriate MIM interaction
69902view19:29, 11 July 2013AlexanderPicoModified title
68019view07:45, 29 June 2013EgonwAdded a missing metabolite identifier.
63163view20:24, 8 May 2013MaintBotUpdating gpml version
59219view20:13, 22 February 2013MaintBotUpdated Ensembl and UniProt data source
59147view03:03, 22 February 2013MaintBotModified title
44991view14:36, 6 October 2011MartijnVanIerselOntology Term : 'cholesterol biosynthetic pathway' added !
43562view21:55, 29 June 2011Samuel SklarSREBP added
41229view23:46, 1 March 2011MaintBotRemoved redundant pathway information and comments
35133view13:55, 11 February 2010ThomasModified description
35132view13:53, 11 February 2010Thomasremoved genmapp comments
35131view13:52, 11 February 2010Thomasadded references
34473view18:34, 10 December 2009MaintBotAutomatic update of empty xrefs
31995view13:15, 14 August 2009MaintBotFixed group labels
21093view11:30, 14 November 2008MaintBot[[Pathway:Homo sapiens:Cholesterol Biosynthesis]] moved to [[Pathway:WP197]]: Moved to stable identifier
14362view15:13, 22 May 2008Pahlesfor some metabolites the type was unknown
14346view13:51, 22 May 2008Pahlesreactions connected
14344view13:40, 22 May 2008PahlesPeriodical save, work in progress
14338view13:29, 22 May 2008Pahlesmetabolites added
14336view13:28, 22 May 2008PahlesPeriodical save, work in progress
14329view13:18, 22 May 2008PahlesPeriodical save, work in progress
14324view13:08, 22 May 2008PahlesPeriodical save, work in progress
14318view12:57, 22 May 2008PahlesPeriodical save, work in progress
13048view11:11, 17 May 2008MaintBotautomated metabolite conversion
7954view13:46, 7 January 2008MaintBot[[Pathway:Human:Cholesterol Biosynthesis]] moved to [[Pathway:Homo sapiens:Cholesterol Biosynthesis]]: Renaming species
7601view16:03, 18 December 2007MaintBotfixed category names
7334view12:42, 4 November 2007MaintBotAdded categories to GPML
19405view22:17, 22 May 2007N.Manteigpml file for [[Human:Cholesterol_Biosynthesis]]
6302view22:17, 22 May 2007M.Liebermangpml file for [[Human:Cholesterol_Biosynthesis]]

External references


View all...
NameTypeDatabase referenceComment
(S)-2,3-EpoxysqualeneMetabolite9029-62-3 (CAS)
7-DehydrocholesterolMetabolite434-16-2 (CAS)
Acetyl-CoAUnknown72-89-9 (CAS)
CYP51A1GeneProduct1595 (Entrez Gene)
CholesterolUnknown57-88-5 (CAS)
DHCR7GeneProduct1717 (Entrez Gene)
DimethylallylpyrophosphateMetabolite358-72-5 (CAS)
FDFT1GeneProduct2222 (Entrez Gene)
FDPSGeneProduct2224 (Entrez Gene)
Geranyl-PPMetabolite6699-20-3 (CAS)
HMGCRGeneProduct3156 (Entrez Gene)
HMGCS1GeneProduct3157 (Entrez Gene)
IDI1GeneProduct3422 (Entrez Gene)
LSSGeneProduct4047 (Entrez Gene)
LanosterinMetabolite79-63-0 (CAS)
LathosterolUnknown80-99-9 (CAS)
MVDGeneProduct4597 (Entrez Gene)
MVKGeneProduct4598 (Entrez Gene)
Mevalonic acid 5-pyrophosphateMetabolite1492-08-6 (CAS)
Mevalonic acid-5PMetabolite73566-35-5 (CAS)
Mevalonic acidMetabolite150-97-0 (CAS)
NSDHLGeneProduct50814 (Entrez Gene) NAD(P) dependent steroid dehydrogenase-like
PMVKGeneProduct10654 (Entrez Gene)
SC4MOLGeneProduct6307 (Entrez Gene)
SC5DLGeneProduct6309 (Entrez Gene)
SQLEGeneProduct6713 (Entrez Gene)
SqualeneUnknown111-02-4 (CAS)
farnesyl pyrophosphateMetabolite13058-04-3 (CAS)
isopentenyl pyrophosphateMetabolite358-71-4 (CAS)

Annotated Interactions

No annotated interactions
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