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)
GGO: 109025269(PHYH)
PON: 100444487(PHYH)
NLE: 100596466(PHYH)
MCC: 695915(PHYH)
MCF: 102126599(PHYH)
CSAB: 103237904(PHYH)
RRO: 104664458(PHYH)
RBB: 108531286(PHYH)
CJC: 100394030(PHYH)
SBQ: 101043326(PHYH)
MMU: 16922(Phyh)
RNO: 114209(Phyh)
CGE: 100774732(Phyh)
NGI: 103749079(Phyh)
HGL: 101717354(Phyh) 106007539
CCAN: 109690084(Phyh)
TUP: 102475212(PHYH)
AML: 100467763(PHYH) 100470234
UMR: 103664176(PHYH) 103682385
ORO: 101386900(PHYH)
FCA: 101098238(PHYH)
PTG: 102957487(PHYH) 102969046(PHYHD1)
AJU: 106971027(PHYH)
BTA: 100300115 281400(PHYH)
BOM: 102270224 102286749(PHYH)
BIU: 109567956(PHYH)
PHD: 102342650(PHYH)
CHX: 102179212(PHYH)
OAS: 101107212(PHYH) 105607887
SSC: 100134962(PHYH)
CFR: 102511029(PHYH)
CDK: 105100655(PHYH)
BACU: 103000323(PHYH)
LVE: 103088031(PHYH)
OOR: 101276116(PHYH)
EPZ: 103567243(PHYH) 103567246
EAI: 106840463
MYB: 102244663(PHYH)
MYD: 102753013(PHYH)
HAI: 109393810(PHYH)
RSS: 109442333 109450697(PHYH)
PALE: 102894373(PHYH)
LAV: 100677071(PHYH)
TMU: 101340562
MDO: 100014074(PHYH)
SHR: 100927877(PHYH)
OAA: 100085334(PHYH)
GGA: 426601(PHYH)
MGP: 100550784(PHYH)
CJO: 107318471(PHYH)
APLA: 101791044(PHYH)
ACYG: 106046211(PHYH)
TGU: 100231512(PHYH)
GFR: 102031343(PHYH)
FAB: 101813875(PHYH)
PHI: 102100009(PHYH)
PMAJ: 107204956(PHYH)
CCW: 104694497(PHYH)
FPG: 101915232(PHYH)
FCH: 102060134(PHYH)
CLV: 102085518(PHYH)
AAM: 106485478(PHYH)
ASN: 102372139(PHYH)
AMJ: 102572271(PHYH)
PSS: 102463722(PHYH)
CMY: 102945710(PHYH)
CPIC: 101936756(PHYH)
ACS: 100560714 100560911(phyh)
PVT: 110071511 110081229(PHYH)
PBI: 103068159(PHYH)
GJA: 107118480(PHYH)
XLA: 108711219(phyh.L) 446332(phyh.S)
XTR: 100379998(phyh)
NPR: 108784804(PHYH)
DRE: 550521(phyh)
SANH: 107658861
SGH: 107584013(phyh)
CCAR: 109066471(phyh)
IPU: 108280036(phyh)
AMEX: 103026834(phyh)
TRU: 101069626(phyh)
LCO: 104930594(phyh)
NCC: 104961091(phyh)
MZE: 101468356(phyh)
OLA: 101162091(phyh)
XMA: 102223740(phyh)
PRET: 103459683(phyh)
NFU: 107381937(phyh)
CSEM: 103383104(phyh)
LCF: 108874301(phyh)
HCQ: 109528844(phyh)
BPEC: 110161769(phyh)
SASA: 100195182(pahx)
ELS: 105007489(phyh)
SFM: 108926656(phyh)
LCM: 102366940(PHYH)
CMK: 103190516(phyh)
CIN: 100180098
SPU: 579037
SOC: 105204439
AEC: 105153832
ACEP: 105624404
PBAR: 105433940
HST: 105182333
CFO: 105248435
LHU: 105680092
PGC: 109855790
NVI: 100118569
TCA: 658138
ZNE: 110833304
FCD: 110843001
CEL: CELE_ZK550.5(ZK550.5) CELE_ZK550.6(ZK550.6)
CBR: CBG00370
CRG: 105338069
MYI: 110464938
OBI: 106874674
LAK: 106170645
EPA: 110254831
ADF: 107343439
HMG: 100210387
ATH: AT2G01490(PAHX)
CRB: 17886344
BRP: 103854067
BOE: 106322865
THJ: 104823806
CPAP: 110813412
CIT: 102611341
TCC: 18609786
GRA: 105785702
GHI: 107939631
EGR: 104416398
VRA: 106766985
VAR: 108342114
CCAJ: 109787702
CAM: 101493765
LJA: Lj4g3v0643880.1(Lj4g3v0643880.1) Lj4g3v0643880.2(Lj4g3v0643880.2)
ADU: 107484164
AIP: 107639257
LANG: 109341393
FVE: 101302927
PPER: 18792103
PMUM: 103341606
PAVI: 110764569
ZJU: 107429322
CSV: 101218922
CMO: 103502875
CMAX: 111493063
CMOS: 111458811
CPEP: 111780472
RCU: 8258202
JCU: 105634069
HBR: 110663207
POP: 7462233
JRE: 108994097
VVI: 100254163
SLY: 101249915
SPEN: 107018501
SOT: 102599620
CANN: 107847498
NSY: 104233686
NTO: 104094447
SIND: 105156242
DCR: 108206333
BVG: 104883814
SOE: 110785673
NNU: 104609962
DOSA: Os03t0708100-01(Os03g0708100) Os05t0473900-01(Os05g0473900)
OBR: 102710110
BDI: 100833021
ATS: 109780746(LOC109780746)
SBI: 8063400
PDA: 103721394
EGU: 105041302
MUS: 103986850
DCT: 110109674
AOF: 109850190
ATR: 18421937
PPP: 112284342
APRO: F751_0106
MAW: MAC_00527
MAJ: MAA_07281
ANI: AN1262.2
ANG: ANI_1_634114(An13g01600)
DFA: DFA_07379
SPAR: SPRG_01670
GPS: C427_2654
GAI: IMCC3135_08210(ectD_1)
HCH: HCH_03217
SALN: SALB1_1200
RSO: RSp0209
RSE: F504_3648
RPI: Rpic_3916
REH: H16_B0166(h16_B0166)
RME: Rmet_3627
CGD: CR3_4468
BMA: BMA3264
BMAL: DM55_466
BMAE: DM78_2737
BMAQ: DM76_447
BMAI: DM57_1919
BMAF: DM51_2859
BMAZ: BM44_3096
BMAB: BM45_367
BPS: BPSL0213
BPSE: BDL_1776
BPSM: BBQ_3217
BPSU: BBN_3339
BPSD: BBX_166
BPK: BBK_1255
BPSH: DR55_876
BPSA: BBU_1929
BPSO: X996_497
BUT: X994_2487
BTE: BTH_I0176
BTQ: BTQ_201
BTJ: BTJ_2284
BTZ: BTL_199
BTD: BTI_3544
BTV: BTHA_256
BTHE: BTN_1193
BTHM: BTRA_400
BTHA: DR62_1368
BTHL: BG87_363
BOK: DM82_3400
BOC: BG90_1418
BVE: AK36_837
BCN: Bcen_2465
BCJ: BCAL0511
BCEN: DM39_3173
BCEW: DM40_682
BCEO: I35_3155
BAM: Bamb_3127
BMU: Bmul_3076
BMJ: BMULJ_00156(phyH)
BMK: DM80_1842
BMUL: NP80_3204
BCT: GEM_0362
BCED: DM42_1931
BDL: AK34_30
BCON: NL30_28495
BUB: BW23_1806
BLAT: WK25_14950
BTEI: WS51_25750
BSEM: WJ12_15430
BPSL: WS57_34195
BMEC: WJ16_15615
BSTG: WT74_16225
BGU: KS03_827
BGO: BM43_1302
BUK: MYA_2840
BUL: BW21_30
BXE: Bxe_A4369
BXB: DR64_2045
BPH: Bphy_0080
BFN: OI25_1342
PPNO: DA70_04875
PPNM: LV28_01385
PPUL: RO07_20330
PSPU: NA29_16880
PAPI: SG18_19445
BPE: BP3605
BPC: BPTD_3551
BPER: BN118_0499
BPET: B1917_3617
BPEU: Q425_38430
BPA: BPP0438
BPAR: BN117_0435
BBR: BB0440
BPT: Bpet4505
BAV: BAV0329
BTRM: SAMEA390648701575(thpD_1)
HAR: HEAR3315
MMS: mma_3549
JAG: GJA_1036(PHYHD1)
CFU: CFU_0471
CARE: LT85_0462
ARC: ABLL_2506
SUN: SUN_0232
PSWU: SY83_07030
CALH: IJ00_06220
PLM: Plim_1638
FMR: Fuma_02735 Fuma_06198(ptlH_3)
 » 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 271:203-11 (1998)
DOI:10.1016/S0009-8981(97)00259-3
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 229:205-10 (1996)
DOI:10.1006/bbrc.1996.1781
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 17:190-3 (1997)
DOI:10.1038/ng1097-190
  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 232:545-51 (1995)
DOI:10.1111/j.1432-1033.1995.545zz.x
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 17:185-9 (1997)
DOI:10.1038/ng1097-185
  Sequence
[hsa:5264] [mmu:16922]
Other DBs
ExplorEnz - The Enzyme Database: 1.14.11.18
IUBMB Enzyme Nomenclature: 1.14.11.18
ExPASy - ENZYME nomenclature database: 1.14.11.18
BRENDA, the Enzyme Database: 1.14.11.18
CAS: 185402-46-4

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