KEGG   ENZYME: 1.17.99.3Help
Entry
EC 1.17.99.3                Enzyme                                 

Name
3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase;
trihydroxycoprostanoyl-CoA oxidase;
THC-CoA oxidase;
THCA-CoA oxidase;
3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA oxidase;
3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oate 24-hydroxylase
Class
Oxidoreductases;
Acting on CH or CH2 groups;
With unknown physiological acceptors
BRITE hierarchy
Sysname
(25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoA:acceptor 24-oxidoreductase (24R-hydroxylating)
Reaction(IUBMB)
(25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoA + H2O + acceptor = (24R,25R)-3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestan-26-oyl-CoA + reduced acceptor [RN:R07374]
Reaction(KEGG)
R07374;
(other) R08735 R08740
Show
Substrate
(25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoA [CPD:C15613];
H2O [CPD:C00001];
acceptor [CPD:C00028]
Product
(24R,25R)-3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestan-26-oyl-CoA [CPD:C15614];
reduced acceptor [CPD:C00030]
Comment
Requires ATP. The reaction in mammals possibly involves dehydrogenation to give a 24(25)-double bond followed by hydration [1]. However, in amphibians such as the Oriental fire-bellied toad (Bombina orientalis), it is probable that the product is formed via direct hydroxylation of the saturated side chain of (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oate and not via hydration of a 24(25) double bond [5]. In microsomes, the free acid is preferred to the coenzyme A ester, whereas in mitochondria, the coenzyme A ester is preferred to the free-acid form of the substrate [1].
History
EC 1.17.99.3 created 2005
Pathway
ec00120  Primary bile acid biosynthesis
ec01100  Metabolic pathways
Orthology
K10214  3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase
Genes
HSA: 8309(ACOX2)
PTR: 460479(ACOX2)
PPS: 100987633(ACOX2)
GGO: 101135855(ACOX2)
PON: 100173031(ACOX2)
NLE: 100579193(ACOX2)
MCC: 701565(ACOX2)
MCF: 102142571(ACOX2)
CSAB: 103227815(ACOX2)
RRO: 104660198(ACOX2)
RBB: 108543617(ACOX2)
CJC: 100404475(ACOX2)
SBQ: 101051802(ACOX2)
MMU: 93732(Acox2)
RNO: 252898(Acox2)
CGE: 100766043(Acox2)
NGI: 103740540(Acox2)
HGL: 101716015(Acox2)
CCAN: 109697261(Acox2)
OCU: 100009549(ACOX2)
TUP: 102491187(ACOX2)
CFA: 100855488(ACOX2)
AML: 100483316(ACOX2)
UMR: 103674276(ACOX2)
ORO: 101386426(ACOX2)
FCA: 101101039(ACOX2)
PTG: 102972085(ACOX2)
AJU: 106968832(ACOX2)
BTA: 514969(ACOX2)
BOM: 102287053(ACOX2)
BIU: 109576197(ACOX2)
PHD: 102318717(ACOX2)
CHX: 102184385(ACOX2)
OAS: 101121460(ACOX2)
SSC: 100516877(ACOX2)
CFR: 102512694(ACOX2)
CDK: 105089810(ACOX2)
BACU: 102999785(ACOX2)
LVE: 103086040(ACOX2)
OOR: 101288466(ACOX2)
ECB: 100050425(ACOX2)
EPZ: 103550743(ACOX2)
EAI: 106848667(ACOX2)
MYB: 102260512(ACOX2)
MYD: 102766328(ACOX2)
HAI: 109388232(ACOX2)
RSS: 109443586(ACOX2)
PALE: 102884254(ACOX2)
LAV: 100655728(ACOX2)
TMU: 101353097
MDO: 100028782(ACOX2)
SHR: 100924634(ACOX2)
OAA: 100074731(ACOX2)
GGA: 416068(ACOX2)
MGP: 100545690(ACOX2)
CJO: 107319785(ACOX2)
APLA: 101804343(ACOX2)
ACYG: 106035476(ACOX2)
TGU: 100228411(ACOX2)
GFR: 102044390(ACOX2)
FAB: 101820029(ACOX2)
PHI: 102102070(ACOX2)
PMAJ: 107210348(ACOX2)
CCAE: 111935016(ACOX2)
CCW: 104683332(ACOX2)
FPG: 101910731(ACOX2)
FCH: 102048175(ACOX2)
CLV: 102092775(ACOX2)
EGZ: 104134791(ACOX2)
AAM: 106489663(ACOX2)
ASN: 102380977(ACOX2)
AMJ: 102563802(ACOX2)
PSS: 102450027(ACOX2)
CMY: 102935400(ACOX2)
CPIC: 101950611(ACOX2)
ACS: 100556696(acox2)
PVT: 110079915(ACOX2)
PBI: 103062545(ACOX2)
GJA: 107119599(ACOX2)
XLA: 414480(acox2.L)
XTR: 548740(acox2)
NPR: 108791853(ACOX2)
LCM: 102363316(ACOX2)
CMK: 103179753(acox2)
CIN: 100186223
 » show all
Taxonomy
Reference
1  [PMID:240854]
  Authors
Gustafsson J.
  Title
Biosynthesis of cholic acid in rat liver. 24-Hydroxylation of 3alpha, 7alpha, 12alpha-trihydroxy-5beta-cholestanoic acid.
  Journal
J Biol Chem 250:8243-7 (1975)
Reference
2  [PMID:2156865]
  Authors
Schepers L, Van Veldhoven PP, Casteels M, Eyssen HJ, Mannaerts GP.
  Title
Presence of three acyl-CoA oxidases in rat liver peroxisomes. An inducible fatty acyl-CoA oxidase, a noninducible fatty acyl-CoA oxidase, and a noninducible trihydroxycoprostanoyl-CoA oxidase.
  Journal
J Biol Chem 265:5242-6 (1990)
Reference
3  [PMID:8856068]
  Authors
Dieuaide-Noubhani M, Novikov D, Baumgart E, Vanhooren JC, Fransen M, Goethals M, Vandekerckhove J, Van Veldhoven PP, Mannaerts GP.
  Title
Further characterization of the peroxisomal 3-hydroxyacyl-CoA dehydrogenases from rat liver. Relationship between the different dehydrogenases and evidence that fatty acids and the C27 bile acids di- and tri-hydroxycoprostanic acids are metabolized by separate multifunctional proteins.
  Journal
Eur J Biochem 240:660-6 (1996)
DOI:10.1111/j.1432-1033.1996.0660h.x
Reference
4
  Authors
Dieuaide-Noubhani, M., Novikov, D., Baumgart, E., Vanhooren, J.C., Fransen, M., Goethals, M., Vandekerckhove, J., Van Veldhoven, P.P. and Mannaerts, G.P.
  Title
Erratum report. Further characterization of the peroxisomal 3-hydroxyacyl-CoA dehydrogenases from rat liver. Relationship between the different dehydrogenases and evidence that fatty acids and the C27 bile acids di- and tri-hydroxycoprostanic acids are metabolized by separate multifunctional proteins.
  Journal
Eur J Biochem 243:537 (1997)
Reference
5  [PMID:9218493]
  Authors
Pedersen JI, Eggertsen G, Hellman U, Andersson U, Bjorkhem I.
  Title
Molecular cloning and expression of cDNA encoding 3alpha,7alpha,12alpha-trihydroxy-5beta-chole stanoyl-CoA oxidase from rabbit liver.
  Journal
J Biol Chem 272:18481-9 (1997)
DOI:10.1074/jbc.272.29.18481
  Sequence
[ocu:100009549]
Reference
6  [PMID:12543708]
  Authors
Russell DW.
  Title
The enzymes, regulation, and genetics of bile acid synthesis.
  Journal
Annu Rev Biochem 72:137-74 (2003)
DOI:10.1146/annurev.biochem.72.121801.161712
Other DBs
ExplorEnz - The Enzyme Database: 1.17.99.3
IUBMB Enzyme Nomenclature: 1.17.99.3
ExPASy - ENZYME nomenclature database: 1.17.99.3
BRENDA, the Enzyme Database: 1.17.99.3
CAS: 119799-47-2

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