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
HSA: 79152(FA2H)
PTR: 454243(FA2H)
PPS: 100977213(FA2H)
GGO: 101131568(FA2H)
PON: 100431217(FA2H)
NLE: 100584268(FA2H)
MCC: 710403(FA2H)
MCF: 101865555(FA2H)
CSAB: 103233144(FA2H)
CATY: 105573955(FA2H)
PANU: 101010115(FA2H)
RRO: 104677832(FA2H)
RBB: 108527428(FA2H)
TFN: 117088048(FA2H)
PTEH: 111536155(FA2H)
CJC: 100387942(FA2H)
SBQ: 101044680(FA2H)
MMUR: 105875443(FA2H)
MMU: 338521(Fa2h)
MCAL: 110300170(Fa2h)
MPAH: 110337634(Fa2h)
RNO: 307855(Fa2h)
MCOC: 116070197(Fa2h)
MUN: 110546038(Fa2h)
CGE: 100771615(Fa2h)
NGI: 103725674(Fa2h)
HGL: 101713306(Fa2h)
CPOC: 100734359(Fa2h)
CCAN: 109696570(Fa2h)
OCU: 100341164(FA2H)
OPI: 101520900(FA2H)
TUP: 102490483(FA2H)
CFA: 610417(FA2H)
VVP: 112915793(FA2H)
VLG: 121500657(FA2H)
AML: 100478009(FA2H)
UMR: 103662058(FA2H)
UAH: 113252488(FA2H)
ORO: 101386543(FA2H)
ELK: 111153000
MPUF: 101670618(FA2H)
EJU: 114200722(FA2H)
MLX: 118023857(FA2H)
FCA: 101089563(FA2H)
PYU: 121009793(FA2H)
PBG: 122494464(FA2H)
PTG: 102963097(FA2H)
PPAD: 109245704(FA2H)
AJU: 106988717(FA2H)
HHV: 120248207(FA2H)
BTA: 516891(FA2H)
BOM: 102265884(FA2H)
BIU: 109573064(FA2H)
BBUB: 102415825(FA2H)
CHX: 102175609(FA2H)
OAS: 101116673(FA2H)
ODA: 120872871(FA2H)
CCAD: 122421190(FA2H)
SSC: 100523966(FA2H)
CFR: 102520009(FA2H)
CBAI: 105078585(FA2H)
CDK: 105097310(FA2H)
LVE: 103080793(FA2H)
OOR: 101283594(FA2H)
DLE: 111179515(FA2H)
PCAD: 102989690(FA2H)
PSIU: 116744710(FA2H)
ECB: 100068800(FA2H)
EPZ: 103560160(FA2H)
EAI: 106836708(FA2H)
MYB: 102257174(FA2H)
MYD: 102769465(FA2H)
MMYO: 118674608(FA2H)
MLF: 102426505(FA2H)
MNA: 107525587(FA2H)
PKL: 118720669(FA2H)
HAI: 109384776(FA2H)
DRO: 112300748(FA2H)
SHON: 118999221(FA2H)
AJM: 119048931(FA2H)
PDIC: 114515473(FA2H)
MMF: 118639185(FA2H)
RFQ: 117035036(FA2H)
PALE: 102898664(FA2H)
PGIG: 120617374(FA2H)
RAY: 107510405(FA2H)
MJV: 108409624(FA2H)
TOD: 119249073(FA2H)
LAV: 100671399(FA2H)
TMU: 101351896
MDO: 100023897(FA2H)
GAS: 123233217(FA2H)
SHR: 100918083(FA2H)
PCW: 110192525(FA2H)
OAA: 100081759(FA2H)
GGA: 415687(FA2H)
PCOC: 116243727(FA2H)
MGP: 100545282(FA2H)
CJO: 107319076(FA2H)
NMEL: 110404534(FA2H)
APLA: 101792989(FA2H)
ACYG: 106033117(FA2H)
TGU: 100220859(FA2H)
LSR: 110476245(FA2H)
SCAN: 103816827(FA2H)
PMOA: 120508071(FA2H)
OTC: 121334080(FA2H)
PRUF: 121348994(FA2H)
GFR: 102041743(FA2H)
FAB: 101821267(FA2H)
PHI: 102114432(FA2H)
PMAJ: 107209613(FA2H)
CCAE: 111934766(FA2H)
CCW: 104687564(FA2H)
ETL: 114063431(FA2H)
FPG: 101919757(FA2H)
FCH: 102060167(FA2H)
CLV: 102083790(FA2H)
EGZ: 104130285(FA2H)
NNI: 104017500(FA2H)
ACUN: 113484552(FA2H)
PADL: 103918118(FA2H)
AAM: 106496337(FA2H)
AROW: 112976877(FA2H)
NPD: 112943200(FA2H)
DNE: 112987464(FA2H)
ASN: 102377552(FA2H)
AMJ: 102564415(FA2H)
CPOO: 109310461(FA2H)
GGN: 109289662(FA2H)
PSS: 102453873(FA2H)
CMY: 102931270(FA2H)
CPIC: 101938712(FA2H)
TST: 117886717(FA2H)
CABI: 116828130(FA2H)
MRV: 120384190(FA2H)
ACS: 100565078(fa2h)
PVT: 110081275(FA2H)
SUND: 121937497(FA2H)
PBI: 103062962(FA2H)
PMUR: 107287368(FA2H)
TSR: 106549577(FA2H)
PGUT: 117670424(FA2H)
VKO: 123025450(FA2H)
PMUA: 114601805(FA2H)
ZVI: 118086363(FA2H)
GJA: 107109075(FA2H)
XLA: 108715475(fa2h.S) 398669(fa2h.L)
XTR: 595069(fa2h)
NPR: 108784272(FA2H)
IPU: 108268815(fa2h)
PHYP: 113546547(fa2h)
AMEX: 103043062(fa2h)
EEE: 113581800(fa2h)
TRU: 101069057(fa2h)
LCO: 104935403(fa2h)
NCC: 104951781(fa2h)
CGOB: 115009537(fa2h)
ELY: 117262450(fa2h)
SLUC: 116057799(fa2h)
ECRA: 117949033(fa2h)
PFLV: 114560191(fa2h)
PPUG: 119196190(fa2h)
MSAM: 119893046(fa2h)
CUD: 121514626(fa2h)
MZE: 101466387(fa2h)
ONL: 100712524(fa2h)
OAU: 116315798(fa2h)
OLA: 101160316(fa2h)
OML: 112152780(fa2h)
XMA: 102236477(fa2h)
XCO: 114133280(fa2h)
XHE: 116709636(fa2h)
PRET: 103466232(fa2h)
GAF: 122836248(fa2h)
CVG: 107103088(fa2h)
CTUL: 119796812(fa2h)
NFU: 107389229(fa2h)
KMR: 108246913(fa2h)
ALIM: 106520216(fa2h)
AOCE: 111582931(fa2h)
CSEM: 103379462(fa2h)
POV: 109643541(fa2h)
SSEN: 122775373(fa2h)
HHIP: 117762872(fa2h)
LCF: 108902292(fa2h)
SDU: 111222502(fa2h)
SLAL: 111658766(fa2h)
XGL: 120793310(fa2h)
HCQ: 109520012(fa2h)
BPEC: 110169379(fa2h)
MALB: 109962491(fa2h)
SASA: 106560925(fa2h) 106573787
ELS: 105018529(fa2h)
SFM: 108926627(fa2h)
PKI: 111859194(fa2h)
LOC: 102688002(fa2h)
CMK: 103185962(fa2h)
BFO: 118418051
BBEL: 109473895
CIN: 100184991
SCLV: 120326472
APLC: 110982180
SKO: 100370733
DME: Dmel_CG30502(fa2h)
DER: 6543337
DSE: 6607996
DSI: Dsimw501_GD10257(Dsim_GD10257)
DAN: 6494810
DSR: 110189303
DPE: 6590654
DMN: 108160556
DWI: 6640368
DGR: 6559605
DAZ: 108617015
DNV: 108654588
DHE: 111597004
DVI: 6626209
CCAT: 101451295
BOD: 106617886
MDE: 101893509
SCAC: 106088196
LCQ: 111677628
ACOZ: 120959527
AARA: 120904718
AAG: 5579114
CPII: 120412362
AME: 726585
ACER: 107993934
BIM: 100747516
BBIF: 117212288
BVK: 117238059
BVAN: 117164743
BTER: 100646084
BPYO: 122566907
CCAL: 108623636
OBB: 114872717
MGEN: 117220785
NMEA: 116432519
CGIG: 122405191
SOC: 105205758
MPHA: 105829424
AEC: 105152161
ACEP: 105625910
PBAR: 105426302
VEM: 105568208
HST: 105188591
DQU: 106744865
CFO: 105256533
FEX: 115232909
LHU: 105671951
PGC: 109854295
OBO: 105281376
PCF: 106789226
PFUC: 122515467
VPS: 122635769
MDL: 103578964
CGLO: 123275324
FAS: 105267733
DAM: 107043268
AGIF: 122855126
CCIN: 107268108
TCA: 662776
DPA: 109533013
AGB: 108912630
LDC: 111514291
NVL: 108561002
APLN: 108737020
PPYR: 116160095
OTU: 111417680
BMOR: 101737056
BMAN: 114239968
MSEX: 115447143
BANY: 112043525
PMAC: 106712918
PPOT: 106102682
PXU: 106117218
PRAP: 110998905
ZCE: 119838151
HAW: 110380041
TNL: 113493435
API: 100164095
AGS: 114120287
RMD: 113547820
BTAB: 109034141
DCI: 103514113
ZNE: 110829148
CSEC: 111872101
CEL: CELE_C25A1.5(fath-1)
CBR: CBG_12227
BMY: BM_BM2356(Bma-fath-1)
TSP: Tsp_11648
PCAN: 112577110
BGT: 106061935
GAE: 121388538
CRG: 105338625
MYI: 110449937
PMAX: 117338225
OBI: 106880340
OSN: 115209434
LAK: 106160544
NVE: 5519525
EPA: 110233012
ATEN: 116300709
ADF: 107352994
AMIL: 114977752
PDAM: 113673940
SPIS: 111329538
DGT: 114516450
HMG: 100204744
MNG: MNEG_4364
SCE: YMR272C(SCS7)
ERC: Ecym_2712
KMX: KLMA_10577(SCS7)
NCS: NCAS_0C00470(NCAS0C00470)
NDI: NDAI_0K00470(NDAI0K00470)
TPF: TPHA_0C04710(TPHA0C04710)
TBL: TBLA_0D03180(TBLA0D03180)
TDL: TDEL_0B00680(TDEL0B00680)
KAF: KAFR_0B03850(KAFR0B03850)
CAL: CAALFM_C404590WA(SCS7)
SLB: AWJ20_4369(SCS7)
NCR: NCU03492
NTE: NEUTE1DRAFT66051(NEUTE1DRAFT_66051)
MGR: MGG_03920
SSCK: SPSK_09400
MAW: MAC_07251
MAJ: MAA_08839
CMT: CCM_01655
BFU: BCIN_03g04110(Bcscs7)
MBE: MBM_02851
ANI: AN0918.2
ANG: ANI_1_1924014(An01g14200)
ABE: ARB_02129
TVE: TRV_07255
PTE: PTT_10175
SPO: SPAC19G12.08(scs7)
CNE: CNK00680
CNB: CNBK2810
TASA: A1Q1_03482
HIR: HETIRDRAFT_481508(fa2h1)
ABP: AGABI1DRAFT116231(AGABI1DRAFT_116231)
ABV: AGABI2DRAFT194616(AGABI2DRAFT_194616)
MGL: MGL_3617
MRT: MRET_0393
DFA: DFA_05848
ABRE: pbN1_22400
NAM: NAMH_1072
GXY: GLX_27450
CHU: CHU_0011
SLI: Slin_3482
DFE: Dfer_2546
FAE: FAES_0412
HSW: Hsw_3945
FLM: MY04_0412
ZPR: ZPR_3652
MPW: MPR_1004
MRO: MROS_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
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