KEGG ENZYME: 2.3.1.298

ENZYME: 2.3.1.298

Entry

EC 2.3.1.298                Enzyme

Name

ultra-long-chain ceramide synthase;
mammalian ceramide synthase 3;
sphingoid base N-ultra-long-chain fatty acyl-CoA transferase;
CERS3 (gene name)

Class

Transferases;
Acyltransferases;
Transferring groups other than aminoacyl groups

Sysname

ultra-long-chain fatty acyl-CoA:sphingoid base N-acyltransferase

Reaction(IUBMB)

an ultra-long-chain fatty acyl-CoA + a sphingoid base = an ultra-long-chain ceramide + CoA

Reaction(KEGG)

(other) R01496 R06517

Substrate

ultra-long-chain fatty acyl-CoA;
sphingoid base

Product

ultra-long-chain ceramide;
CoA [CPD:C00010]

Comment

Mammals have six ceramide synthases that exhibit relatively strict specificity regarding the chain-length of their acyl-CoA substrates. Ceramide synthase 3 (CERS3) is the only enzyme that is active with ultra-long-chain acyl-CoA donors (C28 or longer). It is active in the epidermis, where its products are incorporated into acylceramides. CERS3 also accepts (2R)-2-hydroxy fatty acids and omega-hydroxy fatty acids, and can accept very-long-chain acyl-CoA substrates (see EC 2.3.1.297, very-long-chain ceramide synthase). It can use multiple sphingoid bases including sphinganine, sphingosine, phytosphingosine, and (6R)-6-hydroxysphingosine.

History

EC 2.3.1.298 created 2019

Pathway

ec00600

Sphingolipid metabolism

ec01100

Metabolic pathways

Orthology

K24622

very-long-chain ceramide synthase / ultra-long-chain ceramide synthase

Genes

HSA:	204219(CERS3)

PTR:	453683(CERS3)

PPS:	100994802(CERS3)

GGO:	101128445(CERS3)

PON:	100451535(CERS3)

NLE:	100590821(CERS3)

HMH:	116481441(CERS3)

MCC:	693696(CERS3)

MCF:	102143963(CERS3)

MTHB:

126960139

MNI:	105499260(CERS3)

CSAB:

103231276(CERS3)

CATY:

105593986(CERS3)

PANU:

101007923(CERS3)

TGE:	112628897(CERS3)

MLEU:

105528481(CERS3)

RRO:	104676807(CERS3)

RBB:	108512618(CERS3)

TFN:	117070128(CERS3)

PTEH:

111527358(CERS3)

CANG:

105522836(CERS3)

CJC:	100406591(CERS3)

SBQ:	101042956(CERS3)

CIMI:

108296303(CERS3)

CSYR:

103271544(CERS3)

MMUR:

105871278(CERS3)

LCAT:

123644504(CERS3)

PCOQ:

105808927(CERS3)

OGA:	100941752(CERS3)

MMU:	545975(Cers3)

MCAL:

110299355(Cers3)

MPAH:

110333640(Cers3)

RNO:	499174(Cers3)

MCOC:

116102359(Cers3)

ANU:	117711260(Cers3)

MUN:	110546438(Cers3)

CGE:	100770521(Cers3)

MAUA:

101843120(Cers3)

PLEU:

114695508(Cers3)

MORG:

121450848(Cers3)

MFOT:

126505930

AAMP:

119802861(Cers3)

NGI:	103747188(Cers3)

HGL:	101702770(Cers3)

CPOC:

100712857(Cers3)

CCAN:

109700448(Cers3)

DORD:

105982319(Cers3)

DSP:	122123985(Cers3)

PLOP:

125368509(Cers3)

NCAR:

124976987

OCU:

100337851

OPI:	101520281(CERS3)

TUP:	102475021(CERS3)

GVR:	103586925(CERS3)

CFA:	607925(CERS3)

CLUD:

112653663(CERS3)

VVP:	112923446(CERS3)

VLG:	121489824(CERS3)

NPO:	129510158(CERS3)

AML:	100466254(CERS3)

UMR:	103659988(CERS3)

UAH:	113263637(CERS3)

UAR:	123790831(CERS3)

ELK:

111152682

LLV:

125105651

MPUF:

101687825(CERS3)

MNP:	132029106(CERS3)

MLK:	131835689(CERS3)

NVS:	122893869(CERS3)

ORO:	101381953(CERS3)

EJU:	114223819(CERS3)

ZCA:	113909841(CERS3)

MLX:	118022993(CERS3)

NSU:	110572365(CERS3)

LWW:	102728855(CERS3)

FCA:	101099301(CERS3)

PYU:	121042489(CERS3)

PCOO:

112856057(CERS3)

PBG:	122468302(CERS3)

PVIV:

125168995(CERS3)

LRUF:

124517427

PTG:	102968533(CERS3)

PPAD:

109273347(CERS3)

PUC:

125910240

AJU:

106972088

HHV:	120233673(CERS3)

BTA:	614354(CERS3)

BOM:	102282323(CERS3)

BIU:	109575125(CERS3)

BBUB:

102405478(CERS3)

BBIS:

104992832(CERS3)

CHX:	102169911(CERS3)

OAS:	101110093(CERS3)

BTAX:

128066892(CERS3)

ODA:	120875266(CERS3)

CCAD:

122455117(CERS3)

MBEZ:

129550217(CERS3)

SSC:	100152265(CERS3)

CFR:	102505196(CERS3)

CBAI:

105075152(CERS3)

CDK:	105093450(CERS3)

VPC:	102533284(CERS3)

BACU:

103016103(CERS3)

BMUS:

118890439(CERS3)

LVE:	103082414(CERS3)

OOR:	101287722(CERS3)

DLE:	111187344(CERS3)

PCAD:

102975484(CERS3)

PSIU:

116749338(CERS3)

NASI:

112390989(CERS3)

ECB:	100050135(CERS3)

EPZ:	103561069(CERS3)

EAI:	106839474(CERS3)

MYB:	102242969(CERS3)

MYD:	102759130(CERS3)

MMYO:

118679202(CERS3)

MLF:	102428338(CERS3)

PKL:	118704309(CERS3)

EFUS:

103301954(CERS3)

MNA:	107527642(CERS3)

DRO:	112306075(CERS3)

SHON:

118992022(CERS3)

AJM:	119049767(CERS3)

PDIC:

114488632(CERS3)

PHAS:

123809741(CERS3)

MMF:	118640928(CERS3)

PPAM:

129061294(CERS3)

HAI:	109380354(CERS3)

RFQ:	117018868(CERS3)

PALE:

102889613(CERS3)

PGIG:

120587350(CERS3)

PVP:	105290275(CERS3)

RAY:	107515791(CERS3)

MJV:	108386501(CERS3)

TOD:	119245188(CERS3)

SARA:

101550104(CERS3)

SETR:

126021837(CERS3)

LAV:	100671292(CERS3)

TMU:

101341813

ETF:	101653374(CERS3)

DNM:	101414521(CERS3)

MDO:	100020588(CERS3)

GAS:	123234193(CERS3)

SHR:	100919629(CERS3)

AFZ:

127551337

PCW:	110192586(CERS3)

OAA:	100090484(CERS3)

GGA:	426648(CERS3)

MGP:	104912889(CERS3)

CJO:	107318875(CERS3)

TPAI:

128075248(CERS3)

LMUT:

125698426(CERS3)

NMEL:

110403582(CERS3)

APLA:

101794409(CERS3)

ACYG:

106036468(CERS3)

CATA:

118251930(CERS3)

AFUL:

116493542(CERS3)

TGU:	100223140(CERS3)

LSR:	110482993(CERS3)

SCAN:

103816094(CERS3)

PMOA:

120509089(CERS3)

OTC:	121343550(CERS3)

PRUF:

121354481(CERS3)

GFR:	102036650(CERS3)

FAB:	101818464(CERS3)

OMA:	130257364(CERS3)

PHI:	102104999(CERS3)

PMAJ:

107209495(CERS3)

CCAE:

111934002(CERS3)

CCW:	104683905(CERS3)

CBRC:

103619239(CERS3)

ETL:	114068286(CERS3)

ZAB:	102070635(CERS3)

ACHL:

103806343(CERS3)

SVG:	106852186(CERS3)

HRT:	120758786(CERS3)

FPG:	101915529(CERS3)

FCH:	102054381(CERS3)

CLV:	102090020(CERS3)

EGZ:	104122870(CERS3)

NNI:	104017067(CERS3)

PCRI:

104028856(CERS3)

PLET:

104617867(CERS3)

EHS:	104516365(CERS3)

PCAO:

104046209(CERS3)

ACUN:

113484146(CERS3)

TALA:

104368379(CERS3)

PADL:

103914771(CERS3)

AFOR:

103907329(CERS3)

ACHC:

115341326(CERS3)

HALD:

104317589(CERS3)

HLE:	104834583(CERS3)

AGEN:

126043385

GCL:

127020695

CCRI:

104163289(CERS3)

CSTI:

104562225(CERS3)

CMAC:

104475655(CERS3)

MUI:	104541394(CERS3)

BREG:

104630611(CERS3)

FGA:	104079146(CERS3)

GSTE:

104257033(CERS3)

LDI:	104351475(CERS3)

MNB:	103770554(CERS3)

OHA:	104339289(CERS3)

NNT:	104407072(CERS3)

SHAB:

115612772(CERS3)

DPUB:

104299712(CERS3)

PGUU:

104472433(CERS3)

ACAR:

104524379(CERS3)

CPEA:

104386699(CERS3)

AVIT:

104269993(CERS3)

CVF:	104288238(CERS3)

RTD:	128914661(CERS3)

CUCA:

104062938(CERS3)

TEO:	104383570(CERS3)

BRHI:

104496347(CERS3)

AAM:	106495821(CERS3)

AROW:

112969803(CERS3)

NPD:	112948296(CERS3)

TGT:	104568858(CERS3)

DNE:	112996295(CERS3)

SCAM:

104148166(CERS3)

ASN:	102388749(CERS3)

AMJ:	102575439(CERS3)

CPOO:

109309161(CERS3)

GGN:	109288466(CERS3)

PSS:	102452581(CERS3)

CMY:	102939917(CERS3)

CCAY:

125643716(CERS3)

DCC:	119862540(CERS3)

CPIC:

101947682(CERS3)

TST:	117884533(CERS3)

CABI:

116816623(CERS3)

MRV:	120373108(CERS3)

ACS:	100554835(cers3)

ASAO:

132762562(CERS3)

PVT:	110072978(CERS3)

SUND:

121934958(CERS3)

PBI:	103067220(CERS3)

PMUR:

107283879(CERS3)

CTIG:

120315251(CERS3)

PGUT:

117669869(CERS3)

APRI:

131184918(CERS3)

PTEX:

113441996(CERS3)

NSS:	113415553(CERS3)

VKO:	123030808(CERS3)

PMUA:

114584561

ZVI:	118092579(CERS3)

HCG:	128334716(CERS3)

GJA:

107107422

STOW:

125424565(CERS3)

EMC:	129345544(CERS3)

XLA:	379135(cers3.L) 444162(cers3.S)

XTR:	448139(cers3)

NPR:	108790075(CERS3)

RTEM:

120931148(CERS3)

BBUF:

120985986(CERS3)

BGAR:

122923564 122927517(CERS3)

MUO:	115459908(CERS3)

GSH:	117348292(CERS3)

DRE:	100141367(cers3b) 100332380(cers3a)

SRX:	107719675 107733272 107742325

SANH:

107662258 107662883 107686553 107692405

SGH:	107553816 107559674 107562918

CCAR:

109052074 109087142 109109440

CAUA:

113051920 113052001 113100321 113105753 113118158

CGIB:

127933777 127962031 127976940 128017385

PTET:

122348536(cers3a) 122362568(cers3b)

LROH:

127167976(cers3a) 127180680(cers3b)

OMC:	131524944(cers3b)

PPRM:

120464869(cers3b) 120480136(cers3a)

RKG:	130071289(cers3b) 130074049(cers3a)

MAMB:

125254131(cers3b) 125264462(cers3a)

CIDE:

127500170 127516724

MASI:

127456274

TROS:

130552865(cers3a) 130563292(cers3b)

TDW:	130413452(cers3a) 130435287(cers3b)

MANU:

129419537(cers3b) 129430172(cers3a)

IPU:	108264168(cers3b) 108271085(cers3a)

IFU:	128606364(cers3b) 128614005(cers3a)

PHYP:

113526340 113536588(cers3)

SMEO:

124391432(cers3a) 124395980(cers3b)

TFD:	113649636(cers3b) 113652894(cers3a)

TVC:	132851771(cers3b) 132855313(cers3a)

AMEX:

103027875(cers3) 103029792

CMAO:

118818105(cers3b) 118818889(cers3a)

EEE:	113573945(cers3) 113580530

CHAR:

105891127(cers3a)

TRU:

101066117

TFS:	130536130(cers3b)

TNG:	GSTEN00035593G001

LCO:	104929719(cers3) 104935212

NCC:

104957244

TBEN:

117480207(cers3a) 117491732(cers3b)

CGOB:

115004452 115009955

PGEO:

117440476(cers3a) 117447706(cers3b)

GACU:

117550147(cers3a) 117557662(cers3b)

EMAC:

134863523(cers3a) 134881627(cers3b)

ELY:	117255921(cers3a) 117261833(cers3b)

EFO:	125887456(cers3b) 125905346(cers3a)

PLEP:

121944372(cers3a) 121950432(cers3b)

SLUC:

116045176(cers3a) 116045667(cers3b)

ECRA:

117947619(cers3a) 117948874(cers3b)

ESP:	116684187 116694253(cers3) 116702387

PFLV:

114554884 114560654

GAT:	120809008(cers3b)

PPUG:

119196632(cers3b)

AFB:	129102735 129108617(cers3b)

CLUM:

117728780(cers3a)

PSWI:

130193209(cers3a)

MSAM:

119883380(cers3a) 119901488

SCHU:

122873190(cers3a) 122875282(cers3b)

CUD:	121510734(cers3a) 121515377(cers3b)

ALAT:

119018717(cers3a) 119025088

MZE:	101477435(cers3) 101480781

ONL:	100710960 100711452

OAU:	116313518(cers3b) 116320058(cers3a)

OLA:

111947541

OML:	112151761(cers3b)

CSAI:

133444760(cers3b) 133457048(cers3a)

XMA:	102234103 102236156

XCO:	114143780 114160349

XHE:	116708311 116718874

PRET:

103462267 103466657

PFOR:

103133806 103149196(cers3)

PLAI:

106956231 106959127(cers3)

PMEI:

106915516 106925757(cers3)

GAF:	122835013(cers3b) 122846287(cers3a)

PPRL:

129365265(cers3b)

CVG:	107096435 107102400

CTUL:

119773704(cers3a)

GMU:	124866838(cers3a) 124877958(cers3b)

NFU:

107388829

KMR:	108229836(cers3b) 108250391(cers3a)

ALIM:

106514312(cers3)

NWH:	119416282(cers3b) 119429828(cers3a)

AOCE:

111571929 111581063

MCEP:

125005721(cers3a) 125014337(cers3b)

CSEM:

103378927(cers3)

POV:	109623996 109624784(cers3)

SSEN:

122772881(cers3a) 122775403(cers3b)

HHIP:

117759513(cers3a) 117763801(cers3b)

HSP:	118109828(cers3b) 118112445(cers3a)

SMAU:

118302196(cers3a) 118315359(cers3b)

LCF:	108875121(cers3b) 108894327(cers3a)

SDU:	111220178(cers3) 111228613

SLAL:

111644372 111653318

XGL:	120793324(cers3b) 120796400(cers3a)

HCQ:

109528089

SSCV:

125971080

SBIA:

133501771(cers3b)

PEE:	133402722(cers3b)

PTAO:

133478293(cers3b)

BPEC:

110162568 110169205

MALB:

109951998 109967919

BSPL:

114857840(cers3b)

SJO:	128359424(cers3b) 128363521(cers3a)

SASA:

106562479 106562576

STRU:

115166866 115203458

OTW:	112241699(cers3a)

OMY:	110506178 110517996

OGO:	124035401 124035507(cers3a)

ONE:

115125322

OKI:

109886548

OKE:

118375141

SNH:	120057070(cers3a)

CCLU:

121548345 123481019

ELS:	105014426(cers3)

SFM:	108941195(cers3)

PKI:	111845096(cers3)

AANG:

118215161 118227406(cers3a)

LOC:	102694534(cers3)

PSPA:

121295084(cers3a) 121299274

ARUT:

117427980 117429295

CPLA:

122541102(cers3a)

ESN:

126981078

HAZT:

108664540

BGT:

106074897

MMER:

123532589

» show all

Reference

1 [PMID:16753040]

Authors

Mizutani Y, Kihara A, Igarashi Y

Title

LASS3 (longevity assurance homologue 3) is a mainly testis-specific (dihydro)ceramide synthase with relatively broad substrate specificity.

Journal

Biochem J 398:531-8 (2006)
DOI:10.1042/BJ20060379

Sequence

[mmu:545975]

Reference

2 [PMID:18541923]

Authors

Mizutani Y, Kihara A, Chiba H, Tojo H, Igarashi Y

Title

2-Hydroxy-ceramide synthesis by ceramide synthase family: enzymatic basis for the preference of FA chain length.

Journal

J Lipid Res 49:2356-64 (2008)
DOI:10.1194/jlr.M800158-JLR200

Sequence

[mmu:545975]

Reference

3 [PMID:22038835]

Authors

Jennemann R, Rabionet M, Gorgas K, Epstein S, Dalpke A, Rothermel U, Bayerle A, van der Hoeven F, Imgrund S, Kirsch J, Nickel W, Willecke K, Riezman H, Grone HJ, Sandhoff R

Title

Loss of ceramide synthase 3 causes lethal skin barrier disruption.

Journal

Hum Mol Genet 21:586-608 (2012)
DOI:10.1093/hmg/ddr494

Sequence

[mmu:545975]

Reference

4 [PMID:23826266]

Authors

Mizutani Y, Sun H, Ohno Y, Sassa T, Wakashima T, Obara M, Yuyama K, Kihara A, Igarashi Y

Title

Cooperative Synthesis of Ultra Long-Chain Fatty Acid and Ceramide during Keratinocyte Differentiation.