KEGG   ENZYME: 1.14.18.5Help
Entry
EC 1.14.18.5                Enzyme                                 

Name
sphingolipid C4-monooxygenase;
sphinganine C4-monooxygenase;
sphingolipid C4-hydroxylase;
SUR2 (gene name);
SBH1 (gene name);
SBH2 (gene name);
DEGS2 (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
dihydroceramide,ferrocytochrome b5:oxygen oxidoreductase (C4-hydroxylating)
Reaction(IUBMB)
a dihydroceramide + 2 ferrocytochrome b5 + O2 + 2 H+ = a (4R)-4-hydroxysphinganine ceramide + 2 ferricytochrome b5 + H2O [RN:R06526]
Reaction(KEGG)
R06526;
(other) R06525
Show
Substrate
dihydroceramide [CPD:C12126];
ferrocytochrome b5 [CPD:C00999];
O2 [CPD:C00007];
H+ [CPD:C00080]
Product
(4R)-4-hydroxysphinganine ceramide [CPD:C12145];
ferricytochrome b5 [CPD:C00996];
H2O [CPD:C00001]
Comment
The enzyme, which belongs to the familiy of endoplasmic reticular cytochrome b5-dependent enzymes, is involved in the biosynthesis of sphingolipids in eukaryotes. Some enzymes are bifunctional and also catalyse EC 1.14.19.17, sphingolipid 4-desaturase [4].
History
EC 1.14.18.5 created 2012 as EC 1.14.13.169, transferred 2015 to EC 1.14.18.5
Pathway
ec00600  Sphingolipid metabolism
ec01100  Metabolic pathways
Orthology
K04712  sphingolipid 4-desaturase/C4-monooxygenase
K04713  sphinganine C4-monooxygenase
Genes
HSA: 123099(DEGS2) 8560(DEGS1)
PTR: 453157(DEGS2) 457770(DEGS1)
PPS: 100979702(DEGS2) 100990486(DEGS1)
GGO: 101143812(DEGS2) 101151016(DEGS1)
PON: 100171788(DEGS1) 100444414(DEGS2)
NLE: 100591022(DEGS1) 100606261(DEGS2)
MCC: 702128(DEGS1) 717222(DEGS2)
MCF: 102125533(DEGS1) 102146231(DEGS2)
CSAB: 103229686(DEGS2) 103230017(DEGS1)
RRO: 104654459(DEGS2) 104677277(DEGS1)
RBB: 108528244(DEGS1) 108542338(DEGS2)
CJC: 100404123(DEGS1) 100409287(DEGS2)
SBQ: 101029219(DEGS2) 101034740(DEGS1)
MMU: 13244(Degs1) 619326(9130409I23Rik) 70059(Degs2)
MCAL: 110292749(Degs1) 110307385(Degs2)
MPAH: 110321736(Degs1) 110322299 110324835(Degs2)
RNO: 289322(RGD1564463) 314438(Degs2) 58970(Degs1)
MUN: 110546668(Degs1) 110551777(Degs2) 110559919
NGI: 103726249 103726263(Degs1) 103727650(Degs2)
HGL: 101706415(Degs2) 101722992(Degs1)
CCAN: 109677085(Degs1) 109694353(Degs2)
OCU: 100342774 100345221(DEGS2) 100355393(DEGS1)
TUP: 102484126(DEGS2) 106734250(DEGS1)
CFA: 490395(DEGS1) 490854(DEGS2)
VVP: 112923572(DEGS1) 112930235(DEGS2)
AML: 100472421(DEGS2) 100478672(DEGS1)
UMR: 103667253(DEGS1) 103673578(DEGS2)
UAH: 113262903(DEGS1) 113267276(DEGS2)
ORO: 101365656(DEGS1) 101366847(DEGS2)
FCA: 101080546(DEGS2) 101083993(DEGS1)
PTG: 102972723(DEGS1)
PPAD: 109258915(DEGS1) 109274554(DEGS2)
AJU: 106983185(DEGS1) 113601448(DEGS2)
BTA: 507290(DEGS1) 540994(DEGS2)
BOM: 102267014(DEGS2) 102270794(DEGS1)
BIU: 109570649(DEGS1) 109575437(DEGS2)
BBUB: 102395639(DEGS2) 102405878(DEGS1)
CHX: 102179052(DEGS2) 102181802(DEGS1)
OAS: 100192315(DEGS1) 101118389(DEGS2) 101120481
SSC: 100135677(DEGS1) 100739329(DEGS2)
CFR: 102511645(DEGS1) 102514149(DEGS2)
CDK: 105095393(DEGS2) 105099817(DEGS1)
BACU: 103009449(DEGS1) 103012505(DEGS2)
LVE: 103077083(DEGS1) 103083645 103091108(DEGS2)
OOR: 101269383(DEGS1) 101281613(DEGS2)
DLE: 111176088(DEGS2) 111177510(DEGS1)
PCAD: 102973359(DEGS2) 102974202(DEGS1)
ECB: 100049834(DEGS2) 100055442(DEGS1)
EPZ: 103556478(DEGS1) 103564051(DEGS2)
EAI: 106833179(DEGS1) 106839663(DEGS2)
MYB: 102251258(DEGS2) 102260316(DEGS1)
MYD: 102761894(DEGS2) 102768904(DEGS1)
MNA: 107541612(DEGS2) 107543612(DEGS1)
HAI: 109381071(DEGS2) 109381186(DEGS1)
DRO: 112311909(DEGS2) 112318898(DEGS1)
PALE: 102877713(DEGS2) 102884338(DEGS1)
RAY: 107497563(DEGS2) 107521085(DEGS1)
MJV: 108400774(DEGS2) 108405657(DEGS1)
LAV: 100663518(DEGS1) 100677351(DEGS2)
MDO: 100018776(DEGS1) 100022759(DEGS2)
SHR: 100924041(DEGS1) 100933090(DEGS2)
PCW: 110205533(DEGS1) 110222390(DEGS2)
OAA: 100079563(DEGS2) 100081682(DEGS1) 100088407
GGA: 421327(DEGS1) 423454(DEGS2)
MGP: 100545871(DEGS1) 100547168(DEGS2)
CJO: 107311009(DEGS1) 107315308(DEGS2)
NMEL: 110396071(DEGS1) 110401292(DEGS2)
APLA: 101791732(DEGS2) 101803108(DEGS1)
ACYG: 106031546(DEGS1) 106033723(DEGS2)
TGU: 100219126(DEGS1) 100225864(DEGS2)
LSR: 110481160(DEGS2) 110483217
SCAN: 103819278(DEGS1) 103826431(DEGS2)
GFR: 102044700(DEGS1) 102044947(DEGS2)
FAB: 101817336(DEGS2) 101820925(DEGS1)
PHI: 102110893(DEGS1) 102114412(DEGS2)
PMAJ: 107201542(DEGS1) 107205859(DEGS2)
CCAE: 111926254(DEGS1) 111929821(DEGS2)
CCW: 104685558(DEGS1) 104686450(DEGS2)
ETL: 114064453(DEGS2) 114073706(DEGS1)
FPG: 101918839(DEGS2) 101919611(DEGS1)
FCH: 102048090(DEGS1) 102059577(DEGS2)
CLV: 102084187(DEGS1) 102086116(DEGS2)
EGZ: 104124506(DEGS2) 104124691(DEGS1)
NNI: 104014818(DEGS2) 104019281(DEGS1)
ACUN: 113477792(DEGS1) 113480655(DEGS2)
PADL: 103920395(DEGS2) 103925021(DEGS1)
AAM: 106482702(DEGS1) 106482961(DEGS2)
ASN: 102372084 102373680(DEGS2) 102374259(DEGS1)
AMJ: 102559976(DEGS1) 102573648 106737226(DEGS2)
PSS: 102455413 102456288(DEGS2) 102457738(DEGS1)
CMY: 102936544(DEGS1) 102939815 102941197(DEGS2)
CPIC: 101933091(DEGS1) 101934087(DEGS2) 101944949
ACS: 100551621(degs2) 100551856(degs1) 100560415
PVT: 110070304(DEGS2) 110075366(DEGS1) 110075723
PBI: 103057812(DEGS1) 103058861(DEGS2) 103062400
PMUR: 107291367(DEGS2) 107292031(DEGS1)
TSR: 106543356(DEGS2) 106549644(DEGS1)
GJA: 107116946 107121524(DEGS2) 107121631(DEGS1)
XLA: 108700206 108716481(degs1.L) 108717695(degs1.S) 380539(degs3.L) 446670 446989(degs2.L)
XTR: 100495063(degs2) 394549(degs3) 493214(degs1)
NPR: 108787065(DEGS1) 108791127 108801754(DEGS2)
DRE: 327075(degs1) 402799(degs2)
IPU: 100528134(degs2) 108260864(degs1)
PHYP: 113538582 113544443(degs1)
AMEX: 103035033(degs1) 103039355(degs2)
EEE: 113576663(degs2) 113585484(degs1)
LCO: 104921571(degs2) 104938997(degs1)
NCC: 104957390(degs1) 104960358(degs2)
MZE: 101475928(degs1) 101481400(degs2)
ONL: 100703192(degs2) 100711880(degs1)
OLA: 101158246(degs2) 101175288(degs1)
XMA: 102226056(degs2) 102230059(degs1)
XCO: 114134747(degs2) 114137810(degs1)
PRET: 103458891(degs2) 103476669(degs1)
CVG: 107087559(degs2) 107088921(degs1)
NFU: 107376444 107392298(degs2)
KMR: 108234807(degs2) 108242396(degs1)
ALIM: 106521433(degs2) 106535193
AOCE: 111563648(degs1) 111588537(degs2)
CSEM: 103376871(degs2) 103387773(degs1)
POV: 109628141(degs2) 109639876(degs1)
LCF: 108888113 108894847(degs1)
SDU: 111225737(degs2) 111239209(degs1)
HCQ: 109517137(degs1) 109526113(degs2)
BPEC: 110155422(degs2) 110156995(degs1)
MALB: 109955044(degs2) 109964347(degs1)
ELS: 105011018 105015975(degs2) 105023239(degs1)
SFM: 108941515(degs2) 108942606
PKI: 111849028(degs1) 111852425(degs2)
LCM: 102355762 102357565(DEGS1) 102358925(DEGS2)
CMK: 103175334(degs2) 103181614(degs1)
SPU: 587766
APLC: 110981957
DME: Dmel_CG9078(ifc)
DER: 6540675
DSI: Dsimw501_GD23376(Dsim_GD23376)
DYA: Dyak_GE25800(Dyak_ifc)
DSR: 110185704
DPE: 6588729
DMN: 108162838
DWI: 6642236
DAZ: 108610628
DNV: 115562528
DHE: 111602344
MDE: 101900309
LCQ: 111679645
AAG: 5577178
AALB: 109424709
AME: 100578035
BIM: 100746260
BTER: 100644949
CCAL: 108623330
OBB: 114875777
MPHA: 105834843
AEC: 105146118
ACEP: 105624284
PBAR: 105430648
VEM: 105569807
DQU: 106749129
CFO: 105254886
LHU: 105669721
PGC: 109860326
OBO: 105285916
PCF: 106789465
NVI: 100116927
CSOL: 105365921
MDL: 103580004
TCA: 660778
DPA: 109543045
NVL: 108569904
BMOR: 101744040
PMAC: 106721272
PRAP: 110997933
HAW: 110378518
TNL: 113498537
PXY: 105389100
API: 100162405
DNX: 107165376
AGS: 114125028
RMD: 113556684
BTAB: 109036809
CLEC: 106668058
ZNE: 110832977
FCD: 110853896
PVM: 113806775
TUT: 107366821
DPTE: 113789969
CSCU: 111625553
PTEP: 107449875
CEL: CELE_F33D4.4(F33D4.4) CELE_Y54E5A.1(ttm-5)
BMY: Bm1_54630
TSP: Tsp_03742
PCAN: 112564085
CRG: 105320205
MYI: 110463500
SHX: MS3_00627
PDAM: 113678608
HMG: 100209713
ATH: AT1G14290(SBH2) AT1G69640(SBH1) AT4G04930(DES-1-LIKE)
LJA: Lj2g3v1280020.1(Lj2g3v1280020.1) Lj5g3v1889400.1(Lj5g3v1889400.1) Lj6g3v2043190.1(Lj6g3v2043190.1)
DOSA: Os02t0639600-01(Os02g0639600) Os02t0745700-01(Os02g0745700) Os06t0226950-01(Os06g0226950) Os06t0330400-00(Os06g0330400) Os07t0627700-01(Os07g0627700)
ATS: 109732110(LOC109732110) 109736566(LOC109736566) 109740771(LOC109740771) 109752027(LOC109752027) 109754174(LOC109754174) 109754190(LOC109754190) 109760276(LOC109760276) 109768799(LOC109768799) 109770401(LOC109770401) 109775925(LOC109775925) 109778107(LOC109778107) 109783086(LOC109783086)
APRO: F751_5185
SCE: YDR297W(SUR2)
KMX: KLMA_30430(SUR2) KLMA_40111(dsd1)
NCS: NCAS_0A01360(NCAS0A01360)
NDI: NDAI_0C02160(NDAI0C02160)
TPF: TPHA_0E01350(TPHA0E01350)
TBL: TBLA_0A02900(TBLA0A02900)
TDL: TDEL_0E03130(TDEL0E03130)
KAF: KAFR_0D04510(KAFR0D04510)
PIC: PICST_54788(FAD3) PICST_77904(SUR2)
SLB: AWJ20_3453(SUR2) AWJ20_4618(DES1)
NTE: NEUTE1DRAFT41476(NEUTE1DRAFT_41476) NEUTE1DRAFT56362(NEUTE1DRAFT_56362)
ANG: ANI_1_1368014(An01g10030) ANI_1_294184(An04g01320)
SPO: SPBC3B8.07c(dsd1) SPBC887.15c(sur2)
ABP: AGABI1DRAFT118992(AGABI1DRAFT_118992) AGABI1DRAFT122617(AGABI1DRAFT_122617)
ABV: AGABI2DRAFT187203(AGABI2DRAFT_187203) AGABI2DRAFT206283(AGABI2DRAFT_206283)
DDI: DDB_G0269738(desA)
DFA: DFA_06927(desA)
SMIN: v1.2.004853.t1(symbB.v1.2.004853.t1)
NGD: NGA_0673910(DEGS) NGA_0673920(DEGS)
TCR: 510565.20
SCL: sce3478
BMX: BMS_0949
BID: Bind_0284
MSL: Msil_1351
MSC: BN69_0791
RUL: UC8_47960
CPI: Cpin_5868
FLN: FLA_1786
MUC: MuYL_1054
MGOT: MgSA37_00742(desA)
SLI: Slin_4982
FAE: FAES_4403
PSEZ: HME7025_01475(degS)
IAL: IALB_0269
 » show all
Taxonomy
Reference
1  [PMID:9368039]
  Authors
Haak D, Gable K, Beeler T, Dunn T
  Title
Hydroxylation of Saccharomyces cerevisiae ceramides requires Sur2p and Scs7p.
  Journal
J Biol Chem 272:29704-10 (1997)
DOI:10.1074/jbc.272.47.29704
  Sequence
[sce:YDR297W]
Reference
2  [PMID:9556590]
  Authors
Grilley MM, Stock SD, Dickson RC, Lester RL, Takemoto JY
  Title
Syringomycin action gene SYR2 is essential for sphingolipid 4-hydroxylation in Saccharomyces cerevisiae.
  Journal
J Biol Chem 273:11062-8 (1998)
DOI:10.1074/jbc.273.18.11062
  Sequence
[sce:YDR297W]
Reference
3  [PMID:11297741]
  Authors
Sperling P, Ternes P, Moll H, Franke S, Zahringer U, Heinz E
  Title
Functional characterization of sphingolipid C4-hydroxylase genes from Arabidopsis thaliana.
  Journal
FEBS Lett 494:90-4 (2001)
DOI:10.1016/S0014-5793(01)02332-8
  Sequence
Reference
4  [PMID:11937514]
  Authors
Ternes P, Franke S, Zahringer U, Sperling P, Heinz E
  Title
Identification and characterization of a sphingolipid delta 4-desaturase family.
  Journal
J Biol Chem 277:25512-8 (2002)
DOI:10.1074/jbc.M202947200
  Sequence
[mmu:70059]
Reference
5  [PMID:15063729]
  Authors
Mizutani Y, Kihara A, Igarashi Y
  Title
Identification of the human sphingolipid C4-hydroxylase, hDES2, and its up-regulation during keratinocyte differentiation.
  Journal
FEBS Lett 563:93-7 (2004)
DOI:10.1016/S0014-5793(04)00274-1
  Sequence
[hsa:123099]
Other DBs
ExplorEnz - The Enzyme Database: 1.14.18.5
IUBMB Enzyme Nomenclature: 1.14.18.5
ExPASy - ENZYME nomenclature database: 1.14.18.5
BRENDA, the Enzyme Database: 1.14.18.5

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