KEGG   ENZYME: 1.14.11.18
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
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)
R05722
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)
MCC: 695915(PHYH)
MCF: 102126599(PHYH)
CSAB: 103237904(PHYH)
CATY: 105583866(PHYH)
PANU: 100462930(PHYH)
RRO: 104664458(PHYH)
RBB: 108531286(PHYH)
TFN: 117081977(PHYH)
PTEH: 111550024(PHYH)
CJC: 100394030(PHYH)
SBQ: 101043326(PHYH)
MMUR: 105871998(PHYH)
MMU: 16922(Phyh)
MCAL: 110310225(Phyh)
MPAH: 110333915(Phyh)
RNO: 114209(Phyh)
MCOC: 116082272(Phyh)
MUN: 110552358(Phyh)
CGE: 100774732(Phyh)
PLEU: 114708537(Phyh)
NGI: 103749079(Phyh)
HGL: 101717354(Phyh) 106007539
CPOC: 100721980(Phyh)
CCAN: 109690084(Phyh)
OPI: 101522852(PHYH)
TUP: 102475212(PHYH)
CFA: 478000
VVP: 112912718 112933299(PHYH)
VLG: 121497417(PHYH) 121498121
AML: 100467763(PHYH) 100470234
UMR: 103664176(PHYH) 103682385
ORO: 101386900(PHYH)
ELK: 111155514
MLX: 118021573(PHYH)
FCA: 101098238(PHYH)
PYU: 121014803(PHYH)
PBG: 122472302(PHYH)
PTG: 102957487(PHYH) 102969046(PHYHD1)
PPAD: 109273714(PHYH)
AJU: 106971027(PHYH)
HHV: 120236938(PHYH)
BTA: 100300115 281400(PHYH)
BOM: 102270224 102286749(PHYH)
BIU: 109567956(PHYH)
BBUB: 102397235(PHYH) 102397569
CHX: 102179212(PHYH)
OAS: 101107212(PHYH)
CCAD: 122448246 122448315(PHYH)
SSC: 100134962(PHYH)
CFR: 102511029(PHYH)
CBAI: 105072463(PHYH)
CDK: 105100655(PHYH)
BACU: 103000323(PHYH)
LVE: 103088031(PHYH)
OOR: 101276116(PHYH)
DLE: 111178076(PHYH)
PCAD: 102988147(PHYH)
PSIU: 116749469(PHYH)
EPZ: 103567243(PHYH) 103567246
EAI: 106840463(PHYH)
MYB: 102244663(PHYH)
MYD: 102753013(PHYH)
MMYO: 118677505(PHYH)
MLF: 102418211(PHYH)
MNA: 107530239(PHYH)
PKL: 118727183(PHYH)
HAI: 109393810(PHYH)
DRO: 112322870(PHYH)
SHON: 119000810(PHYH)
AJM: 119048014(PHYH)
PDIC: 114492390(PHYH)
MMF: 118634453(PHYH)
RFQ: 117019131(PHYH)
PALE: 102894373(PHYH)
PGIG: 120614609(PHYH)
PVP: 105291696(PHYH)
RAY: 107504844(PHYH)
MJV: 108385274(PHYH)
TOD: 119244695(PHYH)
LAV: 100677071(PHYH)
TMU: 101340562
MDO: 100014074(PHYH)
GAS: 123248484(PHYH)
SHR: 100927877(PHYH)
PCW: 110217370(PHYH)
OAA: 100085334(PHYH)
GGA: 426601(PHYH)
PCOC: 116225440(PHYH)
MGP: 100550784(PHYH)
CJO: 107318471(PHYH)
NMEL: 110392271(PHYH)
APLA: 101791044(PHYH)
ACYG: 106046211(PHYH)
TGU: 100231512(PHYH)
LSR: 110470932(PHYH)
SCAN: 103813563(PHYH)
PMOA: 120512502(PHYH)
OTC: 121340578(PHYH)
PRUF: 121362347(PHYH)
GFR: 102031343(PHYH)
FAB: 101813875(PHYH)
PHI: 102100009(PHYH)
PMAJ: 107204956(PHYH)
CCAE: 111923112(PHYH)
CCW: 104694497(PHYH)
ETL: 114066630(PHYH)
FPG: 101915232(PHYH)
FCH: 102060134(PHYH)
CLV: 102085518(PHYH)
NNI: 104014617(PHYH)
ACUN: 113491184(PHYH)
TALA: 104366565(PHYH) 116959932
AAM: 106485478(PHYH)
AROW: 112978126(PHYH)
NPD: 112942375(PHYH)
DNE: 112994805(PHYH)
ASN: 102372139(PHYH)
AMJ: 102572271(PHYH)
CPOO: 109315799(PHYH)
GGN: 109292212(PHYH)
PSS: 102463722(PHYH)
CMY: 102945710(PHYH)
CPIC: 101936756(PHYH)
TST: 117874669(PHYH)
CABI: 116835457
MRV: 120395198(PHYH)
ACS: 100560714 100560911(phyh)
PVT: 110081229(PHYH)
SUND: 121931681(PHYH)
PBI: 103068159(PHYH)
PMUR: 107295057(PHYH)
TSR: 106549083(PHYH)
VKO: 123020004(PHYH)
ZVI: 118091093 118091319(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)
CAUA: 113058696(phyh)
IPU: 108280036(phyh)
PHYP: 113532310(phyh)
AMEX: 103026834(phyh)
EEE: 113591541(phyh)
TRU: 101069626(phyh)
LCO: 104930594(phyh)
NCC: 104961091(phyh)
CGOB: 115028103(phyh)
ELY: 117248916(phyh)
PLEP: 121961369(phyh)
SLUC: 116054611(phyh)
ECRA: 117938645(phyh)
PFLV: 114550318(phyh)
GAT: 120816940(phyh)
PPUG: 119210075(phyh)
MSAM: 119909721 119917890(phyh)
CUD: 121505750(phyh)
MZE: 101468356(phyh)
ONL: 100690874(phyh)
OAU: 116317031(phyh)
OLA: 101162091(phyh)
OML: 112139792(phyh)
XMA: 102223740(phyh)
XCO: 114161055(phyh)
XHE: 116736815(phyh)
PRET: 103459683(phyh)
GAF: 122826738(phyh)
CVG: 107096226(phyh)
CTUL: 119794476(phyh)
NFU: 107381937(phyh)
KMR: 108249286(phyh)
ALIM: 106530528(phyh)
AOCE: 111564974(phyh)
CSEM: 103383104(phyh)
POV: 109632674(phyh)
SSEN: 122767283(phyh)
HHIP: 117757548(phyh)
LCF: 108874301(phyh)
SDU: 111225044(phyh)
SLAL: 111667343(phyh)
XGL: 120803372(phyh)
HCQ: 109528844(phyh)
BPEC: 110161769(phyh)
MALB: 109960989(phyh)
SASA: 100195182(pahx)
OMY: 110490340
SALP: 112072726(phyh)
SNH: 120032169(phyh)
ELS: 105007489(phyh)
SFM: 108926656(phyh)
PKI: 111840258(phyh)
AANG: 118218715(phyh)
LOC: 102690477(phyh)
PSPA: 121319842(phyh)
ARUT: 117415108(phyh)
LCM: 102366940(PHYH)
CMK: 103190516(phyh)
RTP: 109928309(phyh)
APLC: 110989925
AME: 410288
ACER: 107996052
BIM: 100749150
BBIF: 117211615
BVK: 117235005
BVAN: 117153236
BTER: 100648165
CCAL: 108625169
OBB: 114882774
MGEN: 117223180
NMEA: 116430568
AGIF: 122849073
ATD: 109595821
AGB: 108909319
BANY: 112047756
DCI: 103520915
HHAL: 106683056
ZNE: 110833304
CSEC: 111864324
DPTE: 113798508
PTEP: 107455720
SDM: 118183906
CEL: CELE_ZK550.5(ZK550.5) CELE_ZK550.6(ZK550.6)
PCAN: 112571426
BGT: 106061655
CRG: 105338069
MYI: 110464938
PMAX: 117331491
OBI: 106874674
OSN: 115216346
ATEN: 116308777
ADF: 107343439
AMIL: 114959929
PDAM: 113685168
SPIS: 111323781
HMG: 100210387
ATH: AT2G01490(PAHX)
ALY: 9311143
CRB: 17886344
BRP: 103854067
BOE: 106322865
THJ: 104823806
CPAP: 110813412
CIT: 102611341
PVY: 116144817
MINC: 123212890
TCC: 18609786
GRA: 105785702
GAB: 108465541
EGR: 104416398
VRA: 106766985
VAR: 108342114
VUN: 114176452
CCAJ: 109787702
APRC: 113858617
CAM: 101493765
LJA: Lj4g3v0643880.1(Lj4g3v0643880.1) Lj4g3v0643880.2(Lj4g3v0643880.2)
ADU: 107484164
AIP: 107639257
AHF: 112741998
LANG: 109341393
FVE: 101302927
RCN: 112196835
PPER: 18792103
PMUM: 103341606
PAVI: 110764569
PDUL: 117636634
ZJU: 107429322
MNT: 21405717
CSV: 101218922
CMO: 103502875
BHJ: 120081638
CMAX: 111493063
CMOS: 111458811
CPEP: 111780472
RCU: 8258202
JCU: 105634069
HBR: 110663207
MESC: 110602183
POP: 7462233
PEU: 105122143
PALZ: 118030744
JRE: 108994097
QLO: 115972416
TWL: 120007781
VVI: 100254163
VRI: 117924907
SLY: 101249915
SPEN: 107018501
SOT: 102599620
CANN: 107847498
NSY: 104233686
NTO: 104094447
NAU: 109235191
INI: 109163311
ITR: 116015244
SIND: 105156242
EGT: 105966065
HAN: 110920919
ECAD: 122596427
LSV: 111890392
CCAV: 112523544
DCR: 108206333
BVG: 104883814
SOE: 110785673
CQI: 110699920
NNU: 104609962
MING: 122076488
DOSA: Os03t0708100-01(Os03g0708100) Os05t0473900-01(Os05g0473900)
OBR: 102710110
BDI: 100833021
ATS: 109780746
SBI: 8063400
PHAI: 112877271
PDA: 103721394
EGU: 105041302
MUS: 103986850
DCT: 110109674
PEQ: 110025823
AOF: 109850190
ATR: 18421937
MNG: MNEG_0138
APRO: F751_0106
CAL: CAALFM_CR09190CA(CaO19.7316)
NCR: NCU04228
NTE: NEUTE1DRAFT56595(NEUTE1DRAFT_56595)
MGR: MGG_06880
MAJ: MAA_07281
MBE: MBM_07890
ANG: ANI_1_304074(An08g01970) ANI_1_634114(An13g01600)
PBN: PADG_12125(PADG_06459)
SMIN: v1.2.010013.t1(symbB.v1.2.010013.t1)
SPAR: SPRG_01670
PAT: Patl_1683
AMAD: I636_19295
AMAI: I635_20150
GNI: GNIT_3616
SDE: Sde_3060
LPN: lpg2694(legD1)
LPH: LPV_3040
LPO: LPO_2975
LPM: LP6_2726(legD1)
LPF: lpl2621
LPP: lpp2748
LPC: LPC_0442
LPA: lpa_03931
LPE: lp12_2687
LFA: LFA_0371
LHA: LHA_2568(Phyhd)
TMC: LMI_2425(Phyhd)
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
BMJ: BMULJ_00156(phyH)
BMU: Bmul_3076
BMK: DM80_1842
BMUL: NP80_3204
BCT: GEM_0362
BCED: DM42_1931
BDL: AK34_30
BCON: NL30_28495
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
CABA: SBC2_00880
BPE: BP3605
BPC: BPTD_3551
BPER: BN118_0499
BPET: B1917_3617
BPEU: Q425_38430
BPAR: BN117_0435
BPA: BPP0438
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
ASUI: ASUIS_2277
ALK: ALEK_2989
AMYT: AMYT_2364
AMAR: AMRN_2399
ACAA: ACAN_2400
AMOL: AMOL_2378
APAI: APAC_2207
HBV: ABIV_0315
HEBR: AEBR_2595
PACO: AACT_2608
ARC: ABLL_2506
SUN: SUN_0232
MXA: MXAN_3973
PSF: PSE_1045
MALU: KU6B_17250
SMAZ: LH19_20290
SHUM: STHU_43390
BMX: BMS_1155
SMAL: SMALA_8372
SALS: SLNWT_0971
SLD: T261_3458
SRW: TUE45_04035(ptlH)
SFK: KY5_8084c
SRJ: SRO_0724
LMOI: VV02_25835
MPH: MLP_16240
FAL: FRAAL2554
PDX: Psed_1312
ACTI: UA75_16685
AMR: AM1_0372
TTR: Tter_1946
PNL: PNK_1871
LCRE: Pla8534_34060(ptlH_2)
AMUC: Pan181_31000(ptlH)
PLM: Plim_1638
PEH: Spb1_24900(ptlH)
PLS: VT03_10780 VT03_23410(ptlH_2)
FMR: Fuma_02735 Fuma_05232(ptlH_2) Fuma_06198(ptlH_3)
GMR: GmarT_08890(ptlH_2)
SDYN: Mal52_54880 Mal52_61010(ptlH_3)
SLI: Slin_4809
NMO: Nmlp_3445
HMU: Hmuk_0567
HALL: LC1Hm_3101(phyH)
 » show all
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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