KEGG ENZYME: 1.14.11.18

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 100462381(PHYH)
NLE:	100596466(PHYH)
MCC:	695915(PHYH)
MCF:	102126599(PHYH)
CSAB:	103237904(PHYH)
RRO:	104664458(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)
OCU:	100338511 100339259 100339756 100344175 100344430 100344679 100345653(PHYHD1) 100356864
TUP:	102475212(PHYH)
CFA:	478000 478001 607509
AML:	100467763(PHYH) 100470234
UMR:	103664176(PHYH) 103682385
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)
BACU:	103000323(PHYH)
LVE:	103088031(PHYH)
ECB:	100056651(PHYH) 100069176
MYB:	102244663(PHYH)
MYD:	102753013(PHYH)
HAI:	109393810(PHYH)
RSS:	109442333 109450697(PHYH)
PALE:	102894373(PHYH)
LAV:	100677071(PHYH)
MDO:	100014074(PHYH)
SHR:	100927877(PHYH)
OAA:	100085334(PHYH)
GGA:	426601(PHYH)
MGP:	100550784(PHYH)
CJO:	107318471(PHYH)
APLA:	101791044(PHYH)
TGU:	100231512(PHYH)
GFR:	102031343(PHYH)
FAB:	101813875(PHYH)
PHI:	102100009(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)
PBI:	103068159(PHYH)
GJA:	107118480(PHYH)
XLA:	108711219(phyh.L) 446332(phyh.S)
XTR:	100379998(phyh)
DRE:	550521(phyh)
IPU:	108280036(phyh)
TRU:	101069626(phyh)
TNG:	GSTEN00013261G001
LCO:	104930594(phyh)
MZE:	101468356(phyh)
OLA:	101162091(phyh)
XMA:	102223740(phyh)
CSEM:	103383104(phyh)
SASA:	100195182(pahx)
LCM:	102366940(PHYH)
CMK:	103190516(phyh)
BFO:	BRAFLDRAFT_215419 BRAFLDRAFT_66696 BRAFLDRAFT_76041
CIN:	100180098
SPU:	579037
SKO:	100367262 100374284
SOC:	105204439
AEC:	105153832
PBAR:	105433940
HST:	105182333
CFO:	105248435
NVI:	100118569
TCA:	658138
BMOR:	101739774 105842603 692832
PXY:	105382794 105387671 105387692 105387694 105389198 105389742 105392830 105396210 105397087
API:	100159854 100165994
PHU:	Phum_PHUM274820
DPX:	DAPPUDRAFT_188919 DAPPUDRAFT_249621 DAPPUDRAFT_316382
ISC:	IscW_ISCW007193 IscW_ISCW007194
CEL:	CELE_ZK550.5(ZK550.5) CELE_ZK550.6(ZK550.6)
CBR:	CBG00370
NAI:	NECAME_13565
HRO:	HELRODRAFT_185000
LGI:	LOTGIDRAFT_202643
CRG:	105338069
OBI:	106874674
NVE:	NEMVE_v1g236889
ADF:	107343439
HMG:	100210387
AQU:	100640946 105313390 105316730
ATH:	AT2G01490(PAHX)
ALY:	ARALYDRAFT_484067
CRB:	CARUB_v10017693mg
EUS:	EUTSA_v10000985mg
BRP:	103854067
BNA:	106388861 106416809 106427988
THJ:	104823806
CIT:	102611341
CIC:	CICLE_v10016178mg
TCC:	18609786
GRA:	105785702
GHI:	107939631
EGR:	104416398
GMX:	100788875 100790934
PVU:	PHAVU_002G095700g
VRA:	106766985
VAR:	108342114
CCAJ:	109787702
MTR:	MTR_4g084560
CAM:	101493765
ADU:	107484164
AIP:	107639257
LJA:	Lj4g3v0643880.1(Lj4g3v0643880.1) Lj4g3v0643880.2(Lj4g3v0643880.2)
LANG:	109341393
FVE:	101302927
PPER:	18792103
PMUM:	103341606
MDM:	103402584 103414220 103452249
PXB:	103931873 103943015
ZJU:	107429322
CSV:	101218922
CMO:	103502875
RCU:	8258202
JCU:	105634069
POP:	POPTR_0006s23940g(POPTRDRAFT_716771)
VVI:	100254163
SLY:	101249915
SPEN:	107018501
SOT:	102599620
INI:	109149707 109163311 109179624
SIND:	105156242
BVG:	104883814
NNU:	104609962
OSA:	4333863 4339087(OsJ_18897)
DOSA:	Os03t0708100-01(Os03g0708100) Os05t0473900-01(Os05g0473900)
OBR:	102710110
BDI:	100833021
ATS:	F775_05856
SBI:	8063400
ZMA:	100273407 103637754 103650819
SITA:	101769463 101770133
PDA:	103721394
EGU:	105041302
MUS:	103986850
ATR:	18421937
SMO:	SELMODRAFT_102227 SELMODRAFT_141696
PPP:	PHYPADRAFT_196385
CRE:	CHLREDRAFT_158296
VCN:	VOLCADRAFT_81992
BPG:	Bathy04g03930
MIS:	MICPUN_86550
MPP:	MICPUCDRAFT_5760
CSL:	COCSUDRAFT_28352
CVR:	CHLNCDRAFT_140522
APRO:	F751_0106
GSL:	Gasu_32140
CCP:	CHC_T00003462001
FOX:	FOXG_15789
NHE:	NECHADRAFT_85877 NECHADRAFT_96363
MAW:	MAC_00527
MAJ:	MAA_07281
CFJ:	CFIO01_01688
ELA:	UCREL1_4661
PSCO:	LY89DRAFT_735074
ANI:	AN1262.2
AFM:	AFUA_1G10170
AOR:	AOR_1_856074(AO090038000507)
ANG:	ANI_1_634114(An13g01600)
AFV:	AFLA_070500
PCS:	Pc22g16220
BCOM:	BAUCODRAFT_434805
NPA:	UCRNP2_727
WSE:	WALSEDRAFT_33062
WIC:	J056_000767
DDI:	DDB_G0278961
DPP:	DICPUDRAFT_74901
DFA:	DFA_07379
ACAN:	ACA1_105970
TET:	TTHERM_01026240
PTM:	GSPATT00006130001 GSPATT00014604001 GSPATT00022358001
FCY:	FRACYDRAFT_160634
AAF:	AURANDRAFT_25217
SPAR:	SPRG_01670
EHX:	EMIHUDRAFT_367094 EMIHUDRAFT_440614
GTT:	GUITHDRAFT_74806
NGR:	NAEGRDRAFT_59944
VNI:	VIBNI_A0779
AMB:	AMBAS45_04990
GPS:	C427_2654
CEB:	B0D95_04365
HJA:	BST95_18735
HCH:	HCH_03217
MME:	Marme_0221
MPC:	Mar181_3384
TSN:	W908_04680
RSO:	RSp0209
RSL:	RPSI07_mp0152
RSN:	RSPO_m00196
RSM:	CMR15_mp10177
RSE:	F504_3648
RPI:	Rpic_3916
RPF:	Rpic12D_4030
RPJ:	N234_21775
RMN:	TK49_19970
RIN:	ACS15_5635
REU:	Reut_B5568
REH:	H16_B0166(h16_B0166)
CNC:	CNE_2c01510
RME:	Rmet_3627
CTI:	RALTA_B0176
CBW:	RR42_s3209
CGD:	CR3_4468
CCUP:	BKK81_21015
BMA:	BMA3264
BMV:	BMASAVP1_A2927
BML:	BMA10229_A2162
BMN:	BMA10247_3421
BMAL:	DM55_466
BMAE:	DM78_2737
BMAQ:	DM76_447
BMAI:	DM57_1919
BMAF:	DM51_2859
BMAZ:	BM44_3096
BMAB:	BM45_367
BPS:	BPSL0213
BPM:	BURPS1710b_0397
BPL:	BURPS1106A_0213
BPD:	BURPS668_0201
BPR:	GBP346_A0115
BPSE:	BDL_1776
BPSM:	BBQ_3217
BPSU:	BBN_3339
BPSD:	BBX_166
BPZ:	BP1026B_I3300
BPQ:	BPC006_I0199
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
BVI:	Bcep1808_3164
BVE:	AK36_837
BUR:	Bcep18194_A6429
BCN:	Bcen_2465
BCH:	Bcen2424_3079
BCM:	Bcenmc03_3097
BCJ:	BCAL0511
BCEN:	DM39_3173
BCEW:	DM40_682
BCEO:	I35_3155
BAM:	Bamb_3127
BAC:	BamMC406_2990
BMU:	Bmul_3076
BMJ:	BMULJ_00156(phyH)
BMK:	DM80_1842
BMUL:	NP80_3204
BCT:	GEM_0362
BCED:	DM42_1931
BCEP:	APZ15_03305
BDL:	AK34_30
BPYR:	ABD05_05440
BCON:	NL30_28495
BUB:	BW23_1806
BDF:	WI26_14910
BLAT:	WK25_14950
BTEI:	WS51_25750
BSEM:	WJ12_15430
BPSL:	WS57_34195
BMEC:	WJ16_15615
BSTG:	WT74_16225
BGL:	bglu_1g33660
BGP:	BGL_1c35520
BGU:	KS03_827
BUG:	BC1001_0095
BGE:	BC1002_0070
BGF:	BC1003_0117
BGD:	bgla_1g37850
BGO:	BM43_1302
BYI:	BYI23_A027220
BUK:	MYA_2840
BUO:	BRPE64_ACDS28410
BUE:	BRPE67_ACDS27720
BUL:	BW21_30
BUQ:	AC233_00710
BPLA:	bpln_1g34490
BUD:	AQ610_17770
BXE:	Bxe_A4369
BXB:	DR64_2045
BPH:	Bphy_0080
BRH:	RBRH_00766
BPX:	BUPH_04000
BPY:	Bphyt_0353
BFN:	OI25_1342
BCAI:	K788_0003461
PSPW:	BJG93_12190
PPK:	U875_12475
PPNO:	DA70_04875
PPNM:	LV28_01385
PRB:	X636_22220
PPUL:	RO07_20330
PSPU:	NA29_16880
PAPI:	SG18_19445
PVE:	UC34_11550
POX:	MB84_15945
PTX:	ABW99_07770
PFG:	AB870_21925
PNR:	AT302_21785
BPE:	BP3605
BPC:	BPTD_3551
BPER:	BN118_0499
BPET:	B1917_3617
BPEU:	Q425_38430
BPA:	BPP0438
BPAR:	BN117_0435
BBR:	BB0440
BBM:	BN115_0427
BBH:	BN112_2973
BBX:	BBS798_0436
BPT:	Bpet4505
BAV:	BAV0329
BHZ:	ACR54_00390
BTRM:	SAMEA390648701575(thpD_1)
BBRO:	BAU06_11915
BFZ:	BAU07_14795
AXY:	AXYL_05883
AXO:	NH44784_027221
AXN:	AX27061_5583
AXS:	LH59_25760
AXX:	ERS451415_05606
ADT:	APT56_25745
AIS:	BUW96_28585
HAR:	HEAR3315
MMS:	mma_3549
JAG:	GJA_1036(PHYHD1)
JAB:	VN23_09465
JAZ:	YQ44_03660
JAL:	BZG29_23875
HSE:	Hsero_3965
HSZ:	ACP92_19795
HHT:	F506_01635
HRB:	Hrubri_3911
CFU:	CFU_0471
CARE:	LT85_0462
CPRA:	CPter91_0551
MNR:	ACZ75_24115
MASW:	AM586_23470
ANT:	Arnit_2688
ARC:	ABLL_2506
ALP:	LPB137_12080
NSA:	Nitsa_0663
SUN:	SUN_0232
SLH:	YH65_09870
CCX:	COCOR_03955
AGE:	AA314_04582
SCU:	SCE1572_33750
SAMY:	DB32_001333
MRM:	A7982_07308
SPHQ:	BWQ93_03375
APB:	SAR116_2366
PMQ:	PM3016_2075
CALH:	IJ00_06220
FGI:	OP10G_0711
PLM:	Plim_1638
PBS:	Plabr_2051
PLS:	VT03_10780
FMR:	Fuma_02735 Fuma_06198(ptlH_3)

» 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 (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] [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

LinkDB

DBGET integrated database retrieval system