KEGG   ENZYME: 1.14.11.18Help
Entry
EC 1.14.11.18               Enzyme                                 

Name
phytanoyl-CoA dioxygenase;
phytanoyl-CoA hydroxylase
Class
Oxidoreductases;
Acting on paired donors, with incorporation or reduction of molecular oxygen;
With 2-oxoglutarate as one donor, and incorporation of one atom of oxygen into each donor
BRITE hierarchy
Sysname
phytanoyl-CoA, 2-oxoglutarate:oxygen oxidoreductase (2-hydroxylating)
Reaction(IUBMB)
phytanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxyphytanoyl-CoA + succinate + CO2 [RN:R05722]
Reaction(KEGG)
Substrate
phytanoyl-CoA [CPD:C02060];
2-oxoglutarate [CPD:C00026];
O2 [CPD:C00007]
Product
2-hydroxyphytanoyl-CoA [CPD:C07343];
succinate [CPD:C00042];
CO2 [CPD:C00011]
Comment
Part of the peroxisomal phytanic acid alpha-oxidation pathway. Requires Fe2+ and ascorbate.
History
EC 1.14.11.18 created 2000
Orthology
K00477  
phytanoyl-CoA hydroxylase
Genes
HSA: 
5264(PHYH)
PTR: 
450310(PHYH)
PPS: 
100983012(PHYH)
PON: 
NLE: 
100596466(PHYH)
MCC: 
695915(PHYH)
MCF: 
102126599(PHYH)
CJC: 
100394030(PHYH)
MMU: 
16922(Phyh)
RNO: 
114209(Phyh)
CGE: 
100774732(Phyh)
NGI: 
103749079(Phyh)
HGL: 
101717354(Phyh)
OCU: 
TUP: 
102475212(PHYH)
CFA: 
AML: 
UMR: 
FCA: 
101098238(PHYH)
PTG: 
102957487(PHYH)
BTA: 
281400(PHYH)
BOM: 
PHD: 
102342650(PHYH)
CHX: 
102179212(PHYH)
OAS: 
SSC: 
100134962(PHYH)
CFR: 
102511029(PHYH)
BACU: 
103000323(PHYH)
LVE: 
103088031(PHYH)
ECB: 
100056651(PHYH)
MYB: 
102244663(PHYH)
MYD: 
102753013(PHYH)
PALE: 
102894373(PHYH)
MDO: 
100014074(PHYH)
SHR: 
100927877(PHYH)
OAA: 
100085334(PHYH)
GGA: 
426601(PHYH)
MGP: 
APLA: 
101791044(PHYH)
TGU: 
100231512(PHYH)
FAB: 
101813875(PHYH)
PHI: 
102100009(PHYH)
FPG: 
101915232(PHYH)
FCH: 
102060134(PHYH)
CLV: 
102085518(PHYH)
ASN: 
AMJ: 
PSS: 
102463722(PHYH)
CMY: 
102945710(PHYH)
ACS: 
PBI: 
103068159(PHYH)
XLA: 
446332(phyh)
XTR: 
100379998(phyh)
DRE: 
550521(phyh)
TRU: 
MZE: 
OLA: 
XMA: 
LCM: 
102366940(PHYH)
CMK: 
103190516(phyh)
BFO: 
CIN: 
SPU: 
NVI: 
TCA: 
BMOR: 
API: 
PHU: 
ISC: 
CEL: 
CELE_ZK550.6(ZK550.6)
CBR: 
HRO: 
LGI: 
NVE: 
HMG: 
AQU: 
NHE: 
MAW: 
MAJ: 
ELA: 
ANI: 
AFM: 
AOR: 
AOR_1_856074(AO090038000507)
ANG: 
ANI_1_634114(An13g01600)
AFV: 
PCS: 
BCOM: 
NPA: 
WSE: 
WIC: 
PTM: 
EHX: 
AMB: 
RSL: 
RSN: 
RSM: 
RSE: 
RPF: 
RPJ: 
REU: 
REH: 
CNC: 
RME: 
CTI: 
BMA: 
BMV: 
BML: 
BMN: 
BMAL: 
BMAE: 
BMAQ: 
BPS: 
BPM: 
BPL: 
BPD: 
BPR: 
BPSE: 
BPSM: 
BPSU: 
BPSD: 
BPZ: 
BPQ: 
BPK: 
BPSH: 
BPSA: 
BTE: 
BTQ: 
BTJ: 
BTZ: 
BTD: 
BTV: 
BTHE: 
BTHM: 
BOK: 
BUT: 
BVI: 
BUR: 
BCN: 
BCH: 
BCM: 
BCJ: 
BCEN: 
BCEW: 
BAM: 
BAC: 
BMU: 
BMJ: 
BMK: 
BCT: 
BCED: 
BXE: 
BXB: 
BPH: 
BPY: 
BGL: 
BUG: 
BGE: 
BGF: 
BRH: 
BGD: 
BYI: 
BUK: 
BPX: 
BUO: 
PPK: 
PPNO: 
PPNM: 
PRB: 
BPE: 
BPC: 
BPER: 
BPA: 
BPAR: 
BBR: 
BBM: 
BBH: 
BPT: 
BAV: 
AXY: 
AXO: 
AXN: 
AXS: 
HAR: 
MMS: 
HSE: 
CFU: 
ANT: 
ARC: 
NSA: 
SUN: 
CCX: 
APB: 
PLM: 
PBS: 
 » show all
Taxonomy
Reference
1  [PMID:9565335]
  Authors
Jansen GA, Mihalik SJ, Watkins PA, Jakobs C, Moser HW, Wanders RJ.
  Title
Characterization of phytanoyl-Coenzyme A hydroxylase in human liver and activity measurements in patients with peroxisomal disorders.
  Journal
Clin. Chim. Acta. 271 (1998) 203-11.
Reference
2  [PMID:8954107]
  Authors
Jansen GA, Mihalik SJ, Watkins PA, Moser HW, Jakobs C, Denis S, Wanders RJ.
  Title
Phytanoyl-CoA hydroxylase is present in human liver, located in peroxisomes, and deficient in Zellweger syndrome: direct, unequivocal evidence for the new, revised pathway of phytanic acid alpha-oxidation in humans.
  Journal
Biochem. Biophys. Res. Commun. 229 (1996) 205-10.
Reference
3  [PMID:9326940]
  Authors
Jansen GA, Ofman R, Ferdinandusse S, Ijlst L, Muijsers AO, Skjeldal OH, Stokke O, Jakobs C, Besley GT, Wraith JE, Wanders RJ.
  Title
Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene.
  Journal
Nat. Genet. 17 (1997) 190-3.
  Sequence
[rno:114209]
Reference
4  [PMID:7556205]
  Authors
Mihalik SJ, Rainville AM, Watkins PA.
  Title
Phytanic acid alpha-oxidation in rat liver peroxisomes. Production of alpha-hydroxyphytanoyl-CoA and formate is enhanced by dioxygenase cofactors.
  Journal
Eur. J. Biochem. 232 (1995) 545-51.
Reference
5  [PMID:9326939]
  Authors
Mihalik SJ, Morrell JC, Kim D, Sacksteder KA, Watkins PA, Gould SJ.
  Title
Identification of PAHX, a Refsum disease gene.
  Journal
Nat. Genet. 17 (1997) 185-9.
  Sequence
[hsa:5264]
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 
CAS: 
185402-46-4

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