KEGG   ENZYME: 1.14.18.6Help
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
BRITE hierarchy
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)
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)
RRO: 
104677832(FA2H)
CJC: 
100387942(FA2H)
MMU: 
338521(Fa2h)
RNO: 
307855(Fa2h)
CGE: 
100771615(Fa2h)
NGI: 
103725674(Fa2h)
HGL: 
101713306(Fa2h)
OCU: 
100341164(FA2H)
TUP: 
102490483(FA2H)
CFA: 
610417(FA2H)
AML: 
100478009(FA2H)
UMR: 
103662058(FA2H)
FCA: 
101089563(FA2H)
PTG: 
102963097(FA2H)
BTA: 
516891(FA2H)
BOM: 
102265884(FA2H)
PHD: 
102342297(FA2H)
CHX: 
102175609(FA2H)
OAS: 
101116673(FA2H)
SSC: 
100523966(FA2H)
CFR: 
102520009(FA2H)
BACU: 
103000454(MLKL)
LVE: 
103080793(FA2H)
ECB: 
100068800(FA2H)
MYB: 
102257174(FA2H)
MYD: 
102769465(FA2H)
PALE: 
102898664(FA2H)
MDO: 
100023897(FA2H)
SHR: 
100918083(FA2H)
OAA: 
100081759(FA2H)
GGA: 
415687(FA2H)
MGP: 
100545282(FA2H)
CJO: 
107319076(FA2H)
APLA: 
101792989(FA2H)
TGU: 
100220859(FA2H)
GFR: 
102041743(FA2H)
FAB: 
101821267(FA2H)
PHI: 
102114432(FA2H)
CCW: 
104687564(FA2H)
FPG: 
101919757(FA2H)
FCH: 
102060167(FA2H)
CLV: 
102083790(FA2H)
AAM: 
106496337(FA2H)
ASN: 
102377552(FA2H)
AMJ: 
102564415(FA2H)
PSS: 
102453873(FA2H)
CMY: 
102931270(FA2H)
ACS: 
100565078(fa2h)
PBI: 
103062962(FA2H)
GJA: 
107109075(FA2H)
XLA: 
398669(fa2h)
XTR: 
595069(fa2h)
TRU: 
MZE: 
OLA: 
101160316(fa2h)
XMA: 
102236477(fa2h)
LCM: 
CMK: 
103185962(fa2h)
BFO: 
CIN: 
SKO: 
DME: 
DPO: 
DAN: 
DER: 
DPE: 
DSE: 
DSI: 
Dsimw501_GD10257(Dsim_GD10257)
DWI: 
DYA: 
Dyak_GE24014(dyak_GLEANR_775)
DGR: 
DMO: 
DVI: 
MDE: 
AAG: 
CQU: 
AME: 
726585(GB16787)
SOC: 
AEC: 
HST: 
CFO: 
TCA: 
BMOR: 
PXY: 
API: 
CEL: 
CELE_C25A1.5(C25A1.5)
CBR: 
BMY: 
LOA: 
TSP: 
LGI: 
CRG: 
OBI: 
NVE: 
HMG: 
TAD: 
MPP: 
CVR: 
SCE: 
YMR272C(SCS7)
AGO: 
ERC: 
KLA: 
LTH: 
VPO: 
ZRO: 
CGR: 
NCS: 
NCAS_0C00470(NCAS0C00470)
NDI: 
NDAI_0K00470(NDAI0K00470)
TPF: 
TPHA_0C04710(TPHA0C04710)
TBL: 
TBLA_0D03180(TBLA0D03180)
TDL: 
TDEL_0B00680(TDEL0B00680)
KAF: 
KAFR_0B03850(KAFR0B03850)
PPA: 
DHA: 
PIC: 
PGU: 
SPAA: 
LEL: 
CAL: 
CTP: 
COT: 
CDU: 
CTEN: 
YLI: 
CLU: 
NCR: 
NTE: 
NEUTE1DRAFT66051(NEUTE1DRAFT_66051)
SMP: 
PAN: 
TTT: 
MTM: 
CTHR: 
MGR: 
TMN: 
FGR: 
FPU: 
NHE: 
TRE: 
MAW: 
MAJ: 
CMT: 
VAL: 
VDA: 
ELA: 
SSL: 
BFU: 
MBE: 
ANI: 
AFM: 
AOR: 
AOR_1_1948174(AO090005001117)
ANG: 
ANI_1_1924014(An01g14200)
AFV: 
ACT: 
NFI: 
PCS: 
CIM: 
CPW: 
PBL: 
PBN: 
URE: 
ABE: 
TVE: 
AJE: 
PNO: 
PTE: 
BZE: 
BSC: 
BOR: 
ZTR: 
PFJ: 
BCOM: 
NPA: 
TML: 
SPO: 
CNE: 
CNB: 
CGI: 
TMS: 
DSQ: 
PCO: 
SHS: 
HIR: 
PSQ: 
ADL: 
FME: 
GTR: 
LBC: 
MRR: 
CCI: 
SCM: 
ABP: 
AGABI1DRAFT116231(AGABI1DRAFT_116231)
ABV: 
AGABI2DRAFT194616(AGABI2DRAFT_194616)
CPUT: 
SLA: 
WSE: 
WIC: 
UMA: 
PFP: 
MGL: 
PGR: 
MLR: 
MBR: 
SRE: 
DDI: 
DPP: 
DFA: 
TET: 
EHX: 
GTT: 
NGR: 
 » show all
Taxonomy
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 (1997) 28281-8.
  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
Yeast. 14 (1998) 311-21.
  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 (2004) 48562-8.
  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 (2005) 245-54.
  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 (2012) 1327-35.
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 

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