KEGG   ENZYME: 1.14.18.6
Entry
EC 1.14.18.6                Enzyme                                 
Name
4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase;
FA2H (gene name);
SCS7 (gene name)
Class
Oxidoreductases;
Acting on paired donors, with incorporation or reduction of molecular oxygen;
With another compound as one donor, and incorporation of one atom of oxygen into the other donor
Sysname
(4R)-4-hydroxysphinganine ceramide,ferrocytochrome-b5:oxygen oxidoreductase (fatty acyl 2-hydroxylating)
Reaction(IUBMB)
a phytoceramide + 2 ferrocytochrome b5 + O2 + 2 H+ = a (2'R)-2'-hydroxyphytoceramide + 2 ferricytochrome b5 + H2O [RN:R11001]
Reaction(KEGG)
R11001
Substrate
phytoceramide [CPD:C12145];
ferrocytochrome b5 [CPD:C00999];
O2 [CPD:C00007];
H+ [CPD:C00080]
Product
(2'R)-2'-hydroxyphytoceramide [CPD:C21020];
ferricytochrome b5 [CPD:C00996];
H2O [CPD:C00001]
Comment
The enzyme, characterized from yeast and mammals, catalyses the hydroxylation of carbon 2 of long- or very-long-chain fatty acids attached to (4R)-4-hydroxysphinganine during de novo ceramide synthesis. The enzymes from yeast and from mammals contain an N-terminal cytochrome b5 domain that acts as the direct electron donor to the desaturase active site. The newly introduced 2-hydroxyl group has R-configuration. cf. EC 1.14.18.7, dihydroceramide fatty acyl 2-hydroxylase.
History
EC 1.14.18.6 created 2015
Orthology
K19703  4-hydroxysphinganine ceramide fatty acyl 2-hydroxylase
Genes
HSA79152(FA2H)
PTR454243(FA2H)
PPS100977213(FA2H)
GGO101131568(FA2H)
PON100431217(FA2H)
NLE100584268(FA2H)
MCC710403(FA2H)
MCF101865555(FA2H)
CSAB103233144(FA2H)
CATY105573955(FA2H)
PANU101010115(FA2H)
TGE112614170(FA2H)
RRO104677832(FA2H)
RBB108527428(FA2H)
TFN117088048(FA2H)
PTEH111536155(FA2H)
CJC100387942(FA2H)
SBQ101044680(FA2H)
CSYR103270192(FA2H)
MMUR105875443(FA2H)
OGA100958392(FA2H)
MMU338521(Fa2h)
MCAL110300170(Fa2h)
MPAH110337634(Fa2h)
RNO307855(Fa2h)
MCOC116070197(Fa2h)
MUN110546038(Fa2h)
CGE100771615(Fa2h)
PLEU114708073 114709106
NGI103725674(Fa2h)
HGL101713306(Fa2h)
CPOC100734359(Fa2h)
CCAN109696570(Fa2h)
DORD105983062(Fa2h)
DSP122126057(Fa2h)
NCAR124967037
OCU100341164(FA2H)
OPI101520900(FA2H)
TUP102490483(FA2H)
CFA610417(FA2H)
VVP112915793(FA2H)
VLG121500657(FA2H)
AML100478009(FA2H)
UMR103662058(FA2H)
UAH113252488(FA2H)
UAR123791306(FA2H)
ELK111153000
LLV125089695
MPUF101670618(FA2H)
ORO101386543(FA2H)
EJU114200722(FA2H)
ZCA113935303(FA2H)
MLX118023857(FA2H)
FCA101089563(FA2H)
PYU121009793(FA2H)
PBG122494464(FA2H)
PTG102963097(FA2H)
PPAD109245704(FA2H)
AJU106988717(FA2H)
HHV120248207(FA2H)
BTA516891(FA2H)
BOM102265884(FA2H)
BIU109573064(FA2H)
BBUB102415825(FA2H)
CHX102175609(FA2H)
OAS101116673(FA2H)
ODA120872871(FA2H)
CCAD122421190(FA2H)
SSC100523966(FA2H)
CFR102520009(FA2H)
CBAI105078585(FA2H)
CDK105097310(FA2H)
VPC102532118(FA2H)
LVE103080793(FA2H)
OOR101283594(FA2H)
DLE111179515(FA2H)
PCAD102989690(FA2H)
PSIU116744710(FA2H)
ECB100068800(FA2H)
EPZ103560160(FA2H)
EAI106836708(FA2H)
MYB102257174(FA2H)
MYD102769465(FA2H)
MMYO118674608(FA2H)
MLF102426505(FA2H)
MNA107525587(FA2H)
PKL118720669(FA2H)
HAI109384776(FA2H)
DRO112300748(FA2H)
SHON118999221(FA2H)
AJM119048931(FA2H)
PDIC114515473(FA2H)
PHAS123805918(FA2H)
MMF118639185(FA2H)
RFQ117035036(FA2H)
PALE102898664(FA2H)
PGIG120617374(FA2H)
PVP105305349(FA2H)
RAY107510405(FA2H)
MJV108409624(FA2H)
TOD119249073(FA2H)
SARA101558197(FA2H)
LAV100671399(FA2H)
TMU101351896
DNM101435200(FA2H)
MDO100023897(FA2H)
GAS123233217(FA2H)
SHR100918083(FA2H)
PCW110192525(FA2H)
OAA100081759(FA2H)
GGA415687(FA2H)
PCOC116243727(FA2H)
MGP100545282(FA2H)
CJO107319076(FA2H)
NMEL110404534(FA2H)
APLA101792989(FA2H)
ACYG106033117(FA2H)
AFUL116493879(FA2H)
TGU100220859(FA2H)
LSR110476245(FA2H)
SCAN103816827(FA2H)
PMOA120508071(FA2H)
OTC121334080(FA2H)
PRUF121348994(FA2H)
GFR102041743(FA2H)
FAB101821267(FA2H)
PHI102114432(FA2H)
PMAJ107209613(FA2H)
CCAE111934766(FA2H)
CCW104687564(FA2H)
ETL114063431(FA2H)
ZAB102066432(FA2H)
FPG101919757(FA2H)
FCH102060167(FA2H)
CLV102083790(FA2H)
EGZ104130285(FA2H)
NNI104017500(FA2H)
ACUN113484552(FA2H)
TALA116960320(FA2H)
PADL103918118(FA2H)
ACHC115345681(FA2H)
AAM106496337(FA2H)
AROW112976877(FA2H)
NPD112943200(FA2H)
DNE112987464(FA2H)
ASN102377552(FA2H)
AMJ102564415(FA2H)
CPOO109310461(FA2H)
GGN109289662(FA2H)
PSS102453873(FA2H)
CMY102931270(FA2H)
CPIC101938712(FA2H)
TST117886717(FA2H)
CABI116828130(FA2H)
MRV120384190(FA2H)
ACS100565078(fa2h)
PVT110081275(FA2H)
SUND121937497(FA2H)
PBI103062962(FA2H)
PMUR107287368(FA2H)
TSR106549577(FA2H)
PGUT117670424(FA2H)
VKO123025450(FA2H)
PMUA114601805(FA2H)
ZVI118086363(FA2H)
GJA107109075(FA2H)
XLA108715475(fa2h.S) 398669(fa2h.L)
XTR595069(fa2h)
NPR108784272(FA2H)
RTEM120916852(FA2H)
BBUF120980470(FA2H)
BGAR122920447(FA2H)
SRX107707407 107707436 107714073
SANH107654164 107674969
SGH107572964 107589178
CCAR109051125 109051187
CAUA113043051 113066797
IPU108268815(fa2h)
PHYP113546547(fa2h)
SMEO124385903(fa2h)
TFD113635876(fa2h)
AMEX103043062(fa2h)
EEE113581800(fa2h)
TRU101069057(fa2h)
TNGGSTEN00014901G001
LCO104935403(fa2h)
NCC104951781(fa2h)
CGOB115009537(fa2h)
ELY117262450(fa2h)
SLUC116057799(fa2h)
ECRA117949033(fa2h)
PFLV114560191(fa2h)
GAT120809582 120812216
PPUG119196190(fa2h)
MSAM119893046(fa2h)
CUD121514626(fa2h)
MZE101466387(fa2h)
ONL100712524(fa2h)
OAU116315798(fa2h)
OLA101160316(fa2h)
OML112152780(fa2h)
XMA102236477(fa2h)
XCO114133280(fa2h)
XHE116709636(fa2h)
PRET103466232(fa2h)
PFOR103142502 103142526
PLAI106937201 106940916
PMEI106907385 106931296
GAF122836248(fa2h)
CVG107103088(fa2h)
CTUL119796812(fa2h)
GMU124882059(fa2h)
NFU107389229(fa2h)
KMR108246913(fa2h)
ALIM106520216(fa2h)
NWH119416166(fa2h)
AOCE111582931(fa2h)
CSEM103379462(fa2h)
POV109643541(fa2h)
SSEN122775373(fa2h)
HHIP117762872(fa2h)
LCF108902292(fa2h)
SDU111222502(fa2h)
SLAL111658766(fa2h)
XGL120793310(fa2h)
HCQ109520012(fa2h)
BPEC110169379(fa2h)
MALB109962491(fa2h)
SASA106560925(fa2h) 106573787
OTW112218447 112252266
OMY110493994 110513138
OGO123992064 124038012(fa2h)
ONE115109296 115134378
SALP111952150 111962278
SNH120053834 120066186
ELS105018529(fa2h)
SFM108926627(fa2h)
PKI111859194(fa2h)
AANG118215231 118227032
LOC102688002(fa2h)
PSPA121296230 121325699
ARUT117424598 117425957
LCM102353320 102362617 102366970 102367411
CMK103185962(fa2h)
RTP109933820 109933823
BFO118418051
BBEL109473895
CIN100184991
SCLV120326472
APLC110982180
SKO100370733
DMEDmel_CG30502(fa2h)
DER6543337
DSE6607996
DSIDsimw501_GD10257(Dsim_GD10257)
DYADyak_GE24014
DAN6494810
DSR110189303
DPODpse_GA24771
DPE6590654
DMN108160556
DWI6640368
DGR6559605
DMODmoj_GI20817
DAZ108617015
DNV108654588
DHE111597004
DVI6626209
CCAT101451295
BOD106617886
MDE101893509
SCAC106088196
LCQ111677628
AGAAgaP_AGAP011282
ACOZ120959527
AARA120904718
AAG5579114
AALB109399104 109409914
CQUCpipJ_CPIJ001291
CPII120412362
CNS116340030
AME726585
ACER107993934
ALAB122719776
BIM100747516
BBIF117212288
BVK117238059
BVAN117164743
BTER100646084
BPYO122566907
CCAL108623636
OBB114872717
MGEN117220785
NMEA116432519
CGIG122405191
SOC105205758
MPHA105829424
AEC105152161
ACEP105625910
PBAR105426302
VEM105568208
HST105188591
DQU106744865
CFO105256533
FEX115232909
LHU105671951
PGC109854295
OBO105281376
PCF106789226
PFUC122515467
VPS122635769
MDL103578964
CGLO123275324
FAS105267733
DAM107043268
AGIF122855126
CCIN107268108
TCA662776
DPA109533013
SOY115874201
ATD109604823 109607940
CSET123306466
AGB108912630
LDC111514291
NVL108561002
APLN108737020
PPYR116160095
OTU111417680
BMOR101737056
BMAN114239968
MSEX115447143
DPLKGM_204118B
BANY112043525
PMAC106712918
PPOT106102682
PXU106117218
PRAP110998905
ZCE119838151
HAW110380041
TNL113493435
OFU114362958
PXY105390474 105394584
API100164095
DNX107166319 107173495
AGS114120287
RMD113547820
BTAB109034141
DCI103514113
NLU111057672 111062460
ZNE110829148
CSEC111872101
CELCELE_C25A1.5(fath-1)
CBRCBG_12227
BMYBM_BM2356(Bma-fath-1)
LOALOAG_18756
NAINECAME_07678
TSPTsp_11648
LGILOTGIDRAFT_195020
PCAN112577110
BGT106061935
GAE121388538
HRF124140868
HRJ124287911
CRG105338625
MYI110449937
PMAX117338225
MMER123548970
OBI106880340
OSN115209434
LAK106160544
NVE5519525
EPA110233012
ATEN116300709
ADF107352994
AMIL114977752
PDAM113673940
SPIS111329538
DGT114516450
XEN124437281
HMG100204744
TADTRIADDRAFT_37592
QSU111983771 112018466
MNGMNEG_4364
BPGBathy12g00660
MPPMICPUCDRAFT_71053
SCEYMR272C(SCS7)
AGOAGOS_AAL183W
ERCEcym_2712
KLAKLLA0_D01639g
KMXKLMA_10577(SCS7)
LTHKLTH0C04114g
VPOKpol_1069p4 Kpol_541p9
ZROZYRO0G13948g
CGRCAGL0F03399g
NCSNCAS_0C00470(NCAS0C00470)
NDINDAI_0K00470(NDAI0K00470)
TPFTPHA_0C04710(TPHA0C04710)
TBLTBLA_0D03180(TBLA0D03180)
TDLTDEL_0B00680(TDEL0B00680)
TGBHG536_0H00690
KAFKAFR_0B03850(KAFR0B03850)
ZMKHG535_0F00380
PPAPAS_chr3_0535
DHADEHA2F00770g DEHA2F07304g
PICPICST_42944
PGUPGUG_01424
SPAASPAPADRAFT_58779
LELLELG_05641
CALCAALFM_C404590WA(SCS7)
CTPCTRG_05735
COTCORT_0B00880
CDUCD36_44280
CTENCANTEDRAFT_116895
YLIYALI0F12749g
CLUCLUG_00673
CLUSA9F13_17g00550
CAURCJI97_002420
SLBAWJ20_4369(SCS7)
PKZC5L36_0C05050
BNNFOA43_004345
BBRXBRETT_002145
NCRNCU03492
NTENEUTE1DRAFT66051(NEUTE1DRAFT_66051)
SMPSMAC_07221
PANPODANSg6973
TTTTHITE_2123151
MTMMYCTH_2145425
CTHRCTHT_0006950
MGRMGG_03920
TMNUCRPA7_8219
SSCKSPSK_09400
FGRFGSG_05164
FPUFPSE_06564
FVRFVEG_04833 FVEG_09980
FOXFOXG_07910
NHENECHADRAFT_68157
TRETRIREDRAFT_1925
TRRM419DRAFT_67306
MAWMAC_07251
MAJMAA_08839
CMTCCM_01655
PLJVFPFJ_06901
VDAVDAG_07018
CFJCFIO01_07473
SAPOSAPIO_CDS2877
ELAUCREL1_9787
PFYPFICI_08814
SSLSS1G_00445
BFUBCIN_03g04110(Bcscs7)
MBEMBM_02851
PSCOLY89DRAFT_768007
GLZGLAREA_03078
ANIAN0918.2
AFMAFUA_1G15820
ACTACLA_019540
NFINFIA_009500
AORAO090005001117
ANGANI_1_1924014(An01g14200)
AFVAFLA_003948
ALUCAKAW2_10225S(SCS7)
ACHEACHE_30734A(SCS7)
APUUAPUU_50879S(SCS7)
PCSPc16g14490
PDPPDIP_65690
TMFPMAA_065990
TRGTRUGW13939_09225
CIMCIMG_03099
CPWCPC735_010470
UREUREG_02856
PBLPAAG_05321
PBNPADG_06664
ABEARB_02129
TVETRV_07255
AJEHCAG_02256
BGHBDBG_07522
PNOSNOG_02392
PTEPTT_10175
BZECOCCADRAFT_8016
BSCCOCSADRAFT_194504
BORCOCMIDRAFT_7790
AALTCC77DRAFT_1010553
ZTRMYCGRDRAFT_69093
PFJMYCFIDRAFT_202482
BCOMBAUCODRAFT_77775
NPAUCRNP2_6014
TMLGSTUM_00001264001
SPOSPAC19G12.08(scs7)
CNECNK00680
CNBCNBK2810
CGICGB_K3610C
TMSTREMEDRAFT_74429
TASAA1Q1_03482
TVSTRAVEDRAFT_151873
DSQDICSQDRAFT_134448
PCOPHACADRAFT_254447
SHSSTEHIDRAFT_119475 STEHIDRAFT_81527
HIRHETIRDRAFT_481508(fa2h1)
PSQPUNSTDRAFT_102352
ADLAURDEDRAFT_110291
FMEFOMMEDRAFT_152706
GTRGLOTRDRAFT_63286
LBCLACBIDRAFT_248952
MRRMoror_7149
CCICC1G_15692
SCMSCHCODRAFT_69904
ABPAGABI1DRAFT116231(AGABI1DRAFT_116231)
ABVAGABI2DRAFT194616(AGABI2DRAFT_194616)
CPUTCONPUDRAFT_129494
SLASERLADRAFT_378265
WSEWALSEDRAFT_59516
WICJ056_002198
UMAUMAG_03647
PFPPFL1_01159
MGLMGL_3617
MRTMRET_0393
MSYMMSY001_1368
PGRPGTG_02316
MLRMELLADRAFT_73617
MBRMONBRDRAFT_15270
SREPTSG_11333
DDIDDB_G0269908
DPPDICPUDRAFT_58510
DFADFA_05848
TGOTGME49_240770
TETTTHERM_00463850 TTHERM_00594230
EHXEMIHUDRAFT_465032
GTTGUITHDRAFT_151008
NGRNAEGRDRAFT_32303
RHDR2APBS1_3578
HYCE5678_04935
ABREpbN1_22400
NAMNAMH_1072
NAPC3L23_05485
CMEDFE773_06975
CPAFC6V80_03830
PCAYFRD00_05985
NPPPP1Y_Lpl541 PP1Y_Mpl11096
NPNJI59_25650
GXYGLX_27450
KREGWK63_04750
BSMBSM4216_3140
GTKGT3570_09865
BLRBRLA_c045060
BRWGOP56_15105
POWIJ21_33540
LFBC1X05_04145
PABSJIR001_02530
AQZKSP35_09870
TPXTurpa_1563
RBARAWN76_009630
HHYHalhy_1608
PCMAY601_0811
PROEH9L23_13375
SHGSph21_3029
AGDFRZ59_12180
OLIFKG96_12945
SBXCA265_13545
CMRCycma_2507
CAMUCA2015_2431
BBDBelba_1617
EVIEchvi_0776
ESTDN752_10235
ECHIFKX85_03515
ALMAO498_01300
MDAIPZ59_06725
CHUCHU_0011
SLISlin_3482
SRDSD10_19465
SMONAWR27_10955
SPIRCWM47_08710
SPIKEXU85_26975
SPIBG8759_27505
STAEHNV11_17345
SRHOHH216_00050
PSEZHME7025_00463
DFEDfer_2546
FAEFAES_0412
FIBA6C57_04765
HSWHsw_3945
HYMN008_09830
HYDPK28_00525
HYEAM218_15525
HYGAUC43_06000
HYPA0257_13955
HYZAXW84_01040
HNVDDQ68_16700
HYHD3Y59_12075
HYJFHG12_09950
HQIH9L05_08580
HRSHER32_11465
HBNGUY19_12795
HTSHMJ29_05735
PKOPKOR_05205
PACTCA264_16000
RUFTH63_15650
RTIDC20_06040
RUDTH61_08385
NIBGU926_14225
FLMMY04_0412
FBTD770_18730
FUVJR347_17490
ZPRZPR_3652
MYRMYRA21_2260
MPWMPR_1004
MODAS202_11040
MYZBK054_10640
AFLAFHG64_05755
ANPFK178_09465
WVIWeevi_1515
EBVF0358_01060
EFALFH779_02990
ESTEHNV03_01330
MROMROS_2240
 » show all
Reference
1  [PMID:9353282]
  Authors
Mitchell AG, Martin CE
  Title
Fah1p, a Saccharomyces cerevisiae cytochrome b5 fusion protein, and its Arabidopsis thaliana homolog that lacks the cytochrome b5 domain both function in the alpha-hydroxylation of sphingolipid-associated very long chain fatty acids.
  Journal
J Biol Chem 272:28281-8 (1997)
DOI:10.1074/jbc.272.45.28281
  Sequence
[ath:AT2G34770]
Reference
2  [PMID:9559540]
  Authors
Dunn TM, Haak D, Monaghan E, Beeler TJ
  Title
Synthesis of monohydroxylated inositolphosphorylceramide (IPC-C) in Saccharomyces cerevisiae requires Scs7p, a protein with both a cytochrome b5-like domain and a hydroxylase/desaturase domain.
  Journal
  Sequence
[sce:YMR272C]
Reference
3  [PMID:15337768]
  Authors
Alderson NL, Rembiesa BM, Walla MD, Bielawska A, Bielawski J, Hama H
  Title
The human FA2H gene encodes a fatty acid 2-hydroxylase.
  Journal
J Biol Chem 279:48562-8 (2004)
DOI:10.1074/jbc.M406649200
  Sequence
[hsa:79152]
Reference
4  [PMID:15658937]
  Authors
Eckhardt M, Yaghootfam A, Fewou SN, Zoller I, Gieselmann V
  Title
A mammalian fatty acid hydroxylase responsible for the formation of alpha-hydroxylated galactosylceramide in myelin.
  Journal
Biochem J 388:245-54 (2005)
DOI:10.1042/BJ20041451
  Sequence
[mmu:338521]
Reference
5  [PMID:22517924]
  Authors
Guo L, Zhang X, Zhou D, Okunade AL, Su X
  Title
Stereospecificity of fatty acid 2-hydroxylase and differential functions of 2-hydroxy fatty acid enantiomers.
  Journal
J Lipid Res 53:1327-35 (2012)
DOI:10.1194/jlr.M025742
Other DBs
ExplorEnz - The Enzyme Database: 1.14.18.6
IUBMB Enzyme Nomenclature: 1.14.18.6
ExPASy - ENZYME nomenclature database: 1.14.18.6
BRENDA, the Enzyme Database: 1.14.18.6

DBGET integrated database retrieval system