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
HGL : 101717354(Phyh) 106007539
NCAR : 124961617 124962141
OCU : 100338511 100339259 100339756 100344175 100344679 100345653(PHYHD1) 100356864
VVP : 112912718 112933299(PHYH)
VLG : 121497417(PHYH) 121498121
AML : 100467763(PHYH) 100470234
UMR : 103664176(PHYH) 103682385
UAR : 123786156(PHYH) 123786332 123789992 123789993
MPUF : 101676398 101676988(PHYH) 101690963
EJU : 114203621 114203622 114210443
ZCA : 113922491(PHYH) 113932647
PTG : 102957487(PHYH) 102969046(PHYHD1)
BTA : 100300115 281400(PHYH)
BOM : 102270224 102286749(PHYH)
BBUB : 102397235(PHYH) 102397569
ODA : 120859242 120859889 120869305(PHYH)
CCAD : 122448246 122448315(PHYH)
EPZ : 103567243(PHYH) 103567246
EAI : 106840463(PHYH) 106840471
TALA : 104366565(PHYH) 116959932
PADL : 103915010 103915028
ACHC : 115341154 115342279
ACS : 100560714 100560911(phyh)
PMUA : 114605031 114605888
ZVI : 118091093 118091319(PHYH)
XLA : 108711219(phyh.L) 446332(phyh.S)
BBUF : 120983419 120983444 120983453 120983478
BGAR : 122925395 122927827 122927828 122927829
MSAM : 119909721 119917890(phyh)
BFO : 118409211 118415139 118415141 118420793
BBEL : 109477446 109477447 109484574
SCLV : 120327023 120337900 120339602 120339603
SKO : 100367262 100374284 100378442
CGIG : 122395221 122398422
SOC : 105204439 105206848 105207428 113005027
MPHA : 105837351 105839891
AEC : 105143947 105145740 105153832
ACEP : 105620509 105624165 105624404
PBAR : 105430195 105433604 105433940
VEM : 105558527 105565031 105569056
HST : 105180958 105181940 105182333
DQU : 106745938 106746344 106749745
CFO : 105248435 105252851 105256842
FEX : 115232836 115237023 115239582 115243840
LHU : 105678173 105679847 105680092
PGC : 109854617 109855790 109858383
OBO : 105281653 105285398 105288037
PFUC : 122515141 122524191
CSOL : 105359733 105360218
TPRE : 106650117 106659374 106660435
MDL : 103568478 103572359 103575757
CGLO : 123260068 123265763
CCIN : 107263505 107268778
LDC : 111502701 111502703 111512626
NVL : 108562900 108563537 108565240 108569762
APLN : 108735674 108740747
PPYR : 116178945 116179329
OTU : 111416644 111416667 111421954 111424645
BMOR : 101739774 105842603 692832
BMAN : 114249838 114249845 114251834
MSEX : 115445497 115448817 115448822 115448824 119191265
DPL : KGM_202988 KGM_214703 KGM_214829 KGM_214830
PMAC : 106707936 106710560
PPOT : 106103279 106110452
PRAP : 110993805 111003851
ZCE : 119828660 119828712 119828787 119835716 119836789
HAW : 110370902 110372496 110382397 110382401
SLIU : 111356654 111363519 111364744 111364832
OFU : 114350014 114360137 114360138 114360145 114360256 114361993
PXY : 105381299 105387671 105387692 105387694 105389198 105389742 105392830 105396210
API : 100159854 100165994 100167257
DNX : 107163652 107163656 107164790
RMD : 113552876 113553779 113553970
CLEC : 106664376 106669420 106669446
FOC : 113206131 113206926 113210888
DPX : DAPPUDRAFT_188919 DAPPUDRAFT_316382 DAPPUDRAFT_321740
DMK : 116916757 116920964 116921193
PCHN : 125026347 125047277
HAME : 121860504 121862768 121875779
PCLA : 123756830 123758616
PTRU : 123507799 123515219
HAZT : 108671571 108673006
ISC : IscW_ISCW007193 IscW_ISCW007194
RSAN : 119406265 119406970
CEL : CELE_ZK550.5(ZK550.5) CELE_ZK550.6(ZK550.6)
HRF : 124113145 124121584 124121599 124121625
HRJ : 124282920 124291902 124291911
MMER : 123528791 123543461
AQU : 100640946 105313390 105316730
CSAT : 104710076 104789908
BNA : 106388861 106416809 106427988
LJA : Lj4g3v0643880.1(Lj4g3v0643880.1) Lj4g3v0643880.2(Lj4g3v0643880.2)
MCHA : 111019644 111022332
SSPL : 121749572 121751336
CSIN : 114261772 114273132
PSOM : 113275529 113282083
NCOL : 116245035 116259651
DOSA : Os03t0708100-01(Os03g0708100) Os05t0473900-01(Os05g0473900)
TAES : 123087133 123090828 123095838
ZMA : 100273407 103637754 103640918 103650819
SITA : 101769463 101770133
PVIR : 120650038 120650040 120654008 120690913 120698518
SMO : SELMODRAFT_102227 SELMODRAFT_141696 SELMODRAFT_416220 SELMODRAFT_440351
CSL : COCSUDRAFT_28352 COCSUDRAFT_37381
MIS : MICPUN_86550 MICPUN_98894
MPP : MICPUCDRAFT_57426 MICPUCDRAFT_5760
CAL : CAALFM_CR09190CA(CaO19.7316)
NTE : NEUTE1DRAFT56595(NEUTE1DRAFT_56595)
FOX : FOXG_15789 FOXG_18742
NHE : NECHADRAFT_77029 NECHADRAFT_85877 NECHADRAFT_94695 NECHADRAFT_96363
PLJ : VFPFJ_06225 VFPFJ_09407 VFPFJ_11236
CFJ : CFIO01_00732 CFIO01_01688 CFIO01_01963
CHIG : CH63R_07972 CH63R_08833
ELA : UCREL1_4246 UCREL1_4661
PFY : PFICI_02180 PFICI_05392 PFICI_14010 PFICI_14715
PSCO : LY89DRAFT_632472 LY89DRAFT_735074 LY89DRAFT_783003
NFI : NFIA_015480 NFIA_034200
AOR : AO090011000425 AO090038000416 AO090038000507
ANG : ANI_1_304074(An08g01970) ANI_1_634114(An13g01600)
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 : Pc16g04850 Pc22g16220
TRG : TRUGW13939_01351 TRUGW13939_09277 TRUGW13939_10076
PBN : PADG_12125(PADG_06459)
BZE : COCCADRAFT_25846 COCCADRAFT_26771
BSC : COCSADRAFT_175888 COCSADRAFT_98957
BOR : COCMIDRAFT_39162 COCMIDRAFT_95850
AALT : CC77DRAFT_1017820 CC77DRAFT_1023029
FFU : CLAFUR5_07557 CLAFUR5_11875
BCOM : BAUCODRAFT_290267 BAUCODRAFT_434805
NPA : UCRNP2_183 UCRNP2_2578 UCRNP2_403 UCRNP2_727
TASA : A1Q1_01035 A1Q1_02921
DPP : DICPUDRAFT_74901 DICPUDRAFT_96115
ACAN : ACA1_105970 ACA1_277850
PTM : GSPATT00006130001 GSPATT00014604001 GSPATT00022358001
SMIN : v1.2.010013.t1(symbB.v1.2.010013.t1)
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)
MYM : A176_002933 A176_002951
CCX : COCOR_03955 COCOR_03959
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
RLI : RLO149_c029630 RLO149_c038650
SAND : H3309_16070 H3309_16075
APB : SAR116_0278 SAR116_1546
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_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_06370 EHS13_06625 EHS13_09575 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
COH : EAV92_03330 EAV92_03840 EAV92_10735
NAH : F5544_10560 F5544_12730
SCB : SCAB_21421 SCAB_8921
SCX : AS200_01265 AS200_06335
SPUN : BFF78_03910 BFF78_11290
SKA : CP970_39720 CP970_43420
SAQU : EJC51_00445 EJC51_41420 EJC51_47100
SPAC : B1H29_00685 B1H29_36375
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
OLE : K0B96_07085 K0B96_12960
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
DFE : Dfer_1903 Dfer_2103 Dfer_2295
» 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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