Entry
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
Product
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
CIMI : 108305268(PHYH) 108309268
HGL : 101717354(Phyh) 106007539
NCAR : 124961617 124962141
MMMA : 107140258 107140446
ITI : 101954510 101958317 101958596 101963034
OCU : 100344175 100344679 100356864 127482758 127485864 127488880 127488881
CLUD : 112658878(PHYH) 112659054
VVP : 112912718 112933299(PHYH)
VLG : 121497417(PHYH) 121498121
AML : 100467763(PHYH) 100470234
UMR : 103664176(PHYH) 103682385
UAH : 125282807 125282808 125282809
UAR : 123786156(PHYH) 123786332 123789992 123789993
MPUF : 101676398 101676988(PHYH) 101690963
MLK : 131838273 131838274 131838275
EJU : 114203621 114203622 114210443
ZCA : 113922491(PHYH) 113932647
BOM : 102270224 102286749(PHYH)
BBUB : 102397235(PHYH) 102397569
BBIS : 105003020 105003022(PHYH)
BTAX : 128052508 128058232(PHYH)
ODA : 120859242 120859889 120869305(PHYH)
CCAD : 122448246 122448315(PHYH)
MREE : 136157807(PHYH) 136159192 136159193
MBEZ : 129557954(PHYH) 129557967
EAI : 106840463(PHYH) 106840471
MDT : 132242277(PHYH) 132242288
MJV : 108385274(PHYH) 108393421
PBRV : 138175845(PHYH) 138175998
CCRI : 104167876(PHYH) 104167878
TALA : 104366565(PHYH) 116959932
ACHC : 115341154 115342279
HALD : 104320261(PHYH) 104320263
HLE : 104840195(PHYH) 104840281
BRHI : 104493623 104493625
PCAO : 104050191 104050192
PADL : 103915010 103915028
AFOR : 103904131 103904158
FGA : 104077080(PHYH) 104077082
PGUU : 104457902 104460960
PLET : 104620584 104623148(PHYH)
SCAM : 104147672(PHYH) 104147685
ACS : 100560714 100560911(phyh)
PGUT : 117657908 117658428 117659233 132712654 132712655
PMUA : 114605031 114605888
PRAF : 128422144 128422147
ZVI : 118091093 118091319(PHYH)
XLA : 108711219(phyh.L) 446332(phyh.S)
BBUF : 120983419 120983444 120983453 120983478
BGAR : 122925395 122927827 122927828 122927829
MUO : 115478397 115478398(PHYH)
PDAB : 135737499 135747008
MSAM : 119909721 119917890(phyh)
STRU : 115180488(phyh) 115198831
CCLU : 121543586 121559068 121559132
PSEX : 120534040 120534566
PMRN : 116938767 116942344(PHYH) 116947152
LRJ : 133345128 133345898 133349497(PHYH)
BFO : 118409211 118415139 118415141 118420793
BBEL : 109477446 109477447 109484574
SCLV : 120327023 120337900 120339602 120339603
LPIC : 129265386 129265387
APLC : 110981039 110989925
ARUN : 117297323 117298665
AAMU : 139943286 139945269
AFIL : 140152615 140163103
SKO : 100367262 100374284 100378442
PFLL : 139135281 139135282 139140095
BCOO : 119075992 119079385 119079386 119085056 119085057
CGIG : 122395221 122398422
SOC : 105204439 105206848 105207428
MPHA : 105837351 105839891
AEC : 105143947 105145740 105153832
ACEP : 105620509 105624165 105624404
PBAR : 105430195 105433604 105433940
VEM : 105558527 105565031 105569056
COBS : 139102597 139105183 139111472(Phyhd1)
TLN : 139813728 139815773(Phyhd1) 139820112
HST : 105180958 105181940 105182333
DQU : 106745938 106746344 106749745
CFO : 105248435 105252851 105256842
FEX : 115232836 115237023 115239582 115243840
PMEC : 138936756 138939965(Phyhd1) 138944981
LHU : 105678173 105679847 105680092
PGC : 109854617 109855790 109858383
OBO : 105281653 105285398 105288037
PFUC : 122515141 122524191
VCRB : 124430790 124431916
CSOL : 105359733 105360218
TPRE : 106650117 106659374 106660435
MDL : 103568478 103572359 103575757
CGLO : 123260068 123265763
CINS : 118067648 118074631
VCAN : 122409363 122409445
CCIN : 107263505 107268778
TMOL : 138123401 138123966
AGRG : 126733512 126747739
LDC : 111502701 111502703 111512626
NVL : 108562900 108563537 108565240 108569762
PPYR : 116178945 116179329
OTU : 111416667 111421954 111424645
BMOR : 101739774 105842603 692832
BMAN : 114249838 114249845 114251834
MSEX : 115445497 115448817 115448822 115448824 119191265
DPL : KGM_214703 KGM_214829 KGM_214830
PMAC : 106707936 106710560
PPOT : 106103279 106110452
BPHI : 137919895 137919925 137924539
PRAP : 110993805 111003851
PNAP : 125049814 125053343 125053344 125053345
ZCE : 119828660 119828712 119828787 119836789
CCRC : 123699649 123699650 123703223
LSIN : 126967470 126968419 126970952
AAGE : 121726021 121727546
HAW : 110370902 110372496 110382397 110382401
HZE : 124639536 124645548 124645550
SLIU : 111356654 111363519 111364744 111364832
OFU : 114350014 114360137 114360138 114360145 114360256 114361993
ONU : 135080441 135083286 135083496 135083497
ATRA : 106136018 106136663 106137563
PXY : 105387671 105389742 105392830
PGW : 126372972 126374879 126377581
LGLY : 125233774 125234141 125234919
CSTB : 134748752 134748760
CCRN : 123293205 123299571 123299572
API : 100159854 100165994 100167257
DNX : 107163652 107163656 107164790
AGS : 114128458 114128644 126549996
RMD : 113552876 113553779 113553970
CLEC : 106669420 106669446
FOC : 113206131 113206926 113210888
BROR : 134532709 134541045 134541285 134541449
DPX : DAPPUDRAFT_188919 DAPPUDRAFT_316382 DAPPUDRAFT_321740
DMK : 116916757 116920964 116921193
DPZ : 124316439 124316451 124328972 124337977
PVM : 113805335 113810973 113811729
PCHN : 125026347 125047277
PMOO : 119580450 119593051
HAME : 121860504 121862768 121875779
PCLA : 123756830 123758616
PTRU : 123507799 123515219
MNZ : 135209033 135209035 135216391 135216808
MRJ : 136833157 136833158 136833713
PORN : 139749194 139758984 139767412
HAZT : 108671571 108673006
PPOI : 119089398 119095395
RSAN : 119406265 119406970
CEL : CELE_ZK550.5(phyh-1) CELE_ZK550.6(ZK550.6)
CRQ : GCK72_012090 GCK72_012091 GCK72_012097
PVUL : 126810793 126811903
HRF : 124113145 124121599 124121625
HRJ : 124282920 124291902 124291911
HASI : 137258583 137293872 137295663 137297114
HCRA : 139444856 139445732 139478133 139478134
CVN : 111107491 111107492 111108762 111108763
MAEA : 128217080 128217367
AQU : 100640946 105313390 105316730
CSAT : 104710076 104789908
BNA : 106388861 106416809 106427988
HSYR : 120133959 120145590
MSYL : 126596628 126609057
PCOX : 137731351 137736837
MCHA : 111019644 111022332
SSPL : 121749572 121751336
CSIN : 114261772 114273132
PSOM : 113275529 113282083
OSA : 4333863(OsJ_12289) 4339087(OsJ_18897)
TAES : 123087133 123090828 123095838
ZMA : 100273407 103637754 103640918 103650819
MFLO : 136451625 136474801 136538366
SITA : 101769463 101770133
PVIR : 120650038 120650040 120654008 120690913 120698518
PAUA : 133913132 133915623
NCOL : 116245035 116259651
SMO : SELMODRAFT_102227 SELMODRAFT_141696 SELMODRAFT_416220 SELMODRAFT_440351
CSL : COCSUDRAFT_28352 COCSUDRAFT_37381
MIS : MICPUN_86550 MICPUN_98894
MPP : MICPUCDRAFT_57426 MICPUCDRAFT_5760
LBG : 92207092(LODBEIA_P18960)
CAL : CAALFM_CR09190CA(CaO19.7316)
CTEN : 18247853(PSN45_000654)
ASAU : 88172799(PUMCH_001734)
YLI : 2906532(YALI2_A00540g)
NTE : NEUTE1DRAFT56595(NEUTE1DRAFT_56595)
SMP : 10800971(SMAC4_04372)
RTHE : 98124605(VTJ83DRAFT_3569)
FVR : FVEG_02958 FVEG_16045
FOX : FOXG_05175 FOXG_15789 FOXG_18742
NHE : NECHADRAFT_40928 NECHADRAFT_77029 NECHADRAFT_85877 NECHADRAFT_94695 NECHADRAFT_96363
FFC : NCS54_00321400 NCS54_00683700 NCS54_00864700 NCS54_01169000 NCS54_01257300
FKR : NCS57_00317200 NCS57_00666000 NCS57_00723600 NCS57_00906800 NCS57_01159500 NCS57_01252100
FMU : J7337_005866 J7337_010759
TRE : TRIREDRAFT_120648(tr43351)
TATV : 90959416(TrAtP1_004029)
TASP : 36615700(TrAFT101_003512)
MAW : 19244838(J3458_003590) 19245438(J3458_004537)
MBRN : 26238833(PHYHD1) 26241045(G6M90_00g097190)
PCHM : VFPPC_03122 VFPPC_09233 VFPPC_16356
PLJ : 28888349(PLICBS_003552) 28891528(PLICBS_010407) 28893352(PLICBS_006488)
PTKZ : JDV02_000294 JDV02_002292 JDV02_008120
CFJ : CFIO01_00732 CFIO01_01688 CFIO01_01963
CLUP : CLUP02_00599 CLUP02_06970 CLUP02_10953
CHIG : CH63R_07972 CH63R_08833
CDET : 87945199(CDEST_08696) 87950009(CDEST_13509)
ELA : UCREL1_4246 UCREL1_4661
PFY : PFICI_02180 PFICI_05392 PFICI_14010 PFICI_14715
PSCO : LY89DRAFT_632472 LY89DRAFT_735074 LY89DRAFT_783003
ANI : ANIA_01262 ANIA_04647
NFI : NFIA_015480 NFIA_034200
AOR : AO090011000425 AO090038000416 AO090038000507
AFV : AFLA_008894 AFLA_008993
ALUC : AKAW2_11689A AKAW2_20056S AKAW2_30790S
ACHE : ACHE_30863S ACHE_80137A
APUU : APUU_50473A APUU_51617S APUU_70935S
PCS : N7525_004766 N7525_011160
TRG : TRUGW13939_01351 TRUGW13939_09277 TRUGW13939_10076
CPW : 9693255(D8B26_000056)
PTRR : 6340428(PtrM4_003670) 6341751(PtrM4_098130)
BZE : COCCADRAFT_25846 COCCADRAFT_26771
BSC : COCSADRAFT_175888 COCSADRAFT_98957
BOR : COCMIDRAFT_39162 COCMIDRAFT_95850
AALT : CC77DRAFT_1017820 CC77DRAFT_1023029
ADAC : 96084482(ACET3X_004160) 96086394(ACET3X_006072)
ARAB : EKO05_0003284 EKO05_0008570
FFU : CLAFUR5_07557 CLAFUR5_11875
CBET : CB0940_07703 CB0940_09717
BCOM : BAUCODRAFT_290267 BAUCODRAFT_434805
NPA : UCRNP2_183 UCRNP2_2578 UCRNP2_403 UCRNP2_727
KNE : 92177970(IAR55_000710)
CCAC : CcaHIS019_0108580(CcaverHIS019_0108580) CcaHIS019_0309490(CcaverHIS019_0309490) CcaHIS019_0410870(CcaverHIS019_0410870)
ABP : AGABI1DRAFT116635(AGABI1DRAFT_116635)
ABV : AGABI2DRAFT196096(AGABI2DRAFT_196096)
SCM : SCHCO_02631857(SCHCODRAFT_02631857)
DPP : DICPUDRAFT_74901 DICPUDRAFT_96115
ACAN : ACA1_105970 ACA1_277850
PTM : GSPATT00006130001 GSPATT00014604001 GSPATT00022358001
AAF : AURANDRAFT_19085 AURANDRAFT_25217 AURANDRAFT_35045
CEG : D0C16_04405 D0C16_11620
GAI : IMCC3135_08210(ectD_1)
REH : H16_B0166(h16_B0166)
PARB : CJU94_04745 CJU94_24745
PFIB : PI93_016120 PI93_021440
BTRM : SAMEA390648701575(thpD_1)
UND : UNDKW_0356 UNDKW_1022(legD1)
UNY : UNDYM_0331 UNDYM_1016(legD1)
RLI : RLO149_c029630 RLO149_c038650
SPJJ : V6U71_05250 V6U71_07625
SAND : H3309_16070 H3309_16075
APB : SAR116_0278 SAR116_1546
SMAB : LN246_01400 LN246_13945
CCX : COCOR_03955 COCOR_03959
PPAK : JY651_13295 JY651_36810
SUR : STAUR_1565 STAUR_4442
AGE : AA314_04582 AA314_05809
AVM : JQX13_20915 JQX13_43030
CFUS : CYFUS_005421 CYFUS_008936
MRM : A7982_07308 A7982_07418
OLE : K0B96_07085 K0B96_12960
MTAR : DF168_00021 DF168_00673 DF168_01759
LCRE : Pla8534_34060(ptlH_2)
PND : Pla175_38870 Pla175_38880
PLS : VT03_10780 VT03_23410(ptlH_2)
FMR : Fuma_02735 Fuma_05232(ptlH_2) Fuma_06198(ptlH_3)
GIM : F1728_09860 F1728_13305
SDYN : Mal52_54880 Mal52_61010(ptlH_3)
PLON : Pla110_33350 Pla110_40360
PHM : PSMK_06950 PSMK_07800 PSMK_08800 PSMK_08900
AGI : FSB73_20315 FSB73_21145
MEKU : HUW50_07160 HUW50_23165 HUW50_23745
PJD : Pjdr2_0510 Pjdr2_1911 Pjdr2_2976 Pjdr2_5210
GYM : GYMC10_2713 GYMC10_2738 GYMC10_3361
PMS : KNP414_01728 KNP414_01778 KNP414_02396
PMQ : PM3016_2025 PM3016_2075 PM3016_2656
PMW : B2K_10555 B2K_10760 B2K_13455
POW : IJ21_29340 IJ21_30680 IJ21_38500 IJ21_44820 IJ21_45380
PSWU : SY83_04060 SY83_07030 SY83_19975 SY83_21005 SY83_21030 SY83_22010
PIB : BBD41_00725 BBD41_27265
PLW : D5F53_13475 D5F53_16095 D5F53_16180
PPSC : EHS13_05125 EHS13_05855 EHS13_06625 EHS13_10435 EHS13_14545 EHS13_21085 EHS13_26990 EHS13_27845 EHS13_29605
PALB : EJC50_00445 EJC50_08270 EJC50_09060 EJC50_09320 EJC50_09595 EJC50_12405 EJC50_13740 EJC50_23770 EJC50_24265 EJC50_24640 EJC50_27225 EJC50_28970
PCHI : PC41400_06475 PC41400_06480 PC41400_06580
PBK : Back11_04190 Back11_20610 Back11_33370 Back11_54590 Back11_61480
PRZ : GZH47_06330 GZH47_09410 GZH47_09625 GZH47_13820 GZH47_15480 GZH47_20260 GZH47_21305 GZH47_22370 GZH47_26040 GZH47_27055 GZH47_31200
PLYC : GXP70_01565 GXP70_06785 GXP70_07000 GXP70_09925 GXP70_20600 GXP70_21210 GXP70_21600 GXP70_22570 GXP70_24080 GXP70_26375 GXP70_26430
PSPN : L1F29_09210 L1F29_22450 L1F29_26115
COH : EAV92_03330 EAV92_03840 EAV92_10735
NAH : F5544_10560 F5544_12730
SCB : SCAB_21421 SCAB_8921
SPUN : BFF78_03910 BFF78_11290
SAQU : EJC51_00445 EJC51_41420 EJC51_47100
SJN : RI060_02595 RI060_04675
BFA : Bfae_28650 Bfae_29130
BRX : BH708_11055 BH708_11510
BGG : CFK41_01795 CFK41_02070
MIK : FOE78_00575 FOE78_08940 FOE78_11905 FOE78_17155 FOE78_21020
MICG : GJV80_01725 GJV80_02290 GJV80_08340 GJV80_19260
KFL : Kfla_2368 Kfla_5005 Kfla_5893
KQI : F1D05_07350 F1D05_14035 F1D05_31800
NOA : BKM31_03910 BKM31_25625
RUB : GBA63_13195 GBA63_13235
CCZ : CCALI_01094 CCALI_01282 CCALI_01760 CCALI_01825 CCALI_02204 CCALI_02303 CCALI_02835
FGI : OP10G_0526 OP10G_0989 OP10G_1123 OP10G_3472
» show all
Taxonomy
Reference
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
Reference
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
Reference
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
Sequence
Reference
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
Reference
Authors
Mihalik SJ, Morrell JC, Kim D, Sacksteder KA, Watkins PA, Gould SJ.
Title
Identification of PAHX, a Refsum disease gene.
Journal
Sequence
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