KEGG   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
HSA204219(CERS3)
PTR453683(CERS3)
PPS100994802(CERS3)
GGO101128445(CERS3)
PON100451535(CERS3)
NLE100590821(CERS3)
MCC693696(CERS3)
MCF102143963(CERS3)
CSAB103231276(CERS3)
CATY105593986(CERS3)
PANU101007923(CERS3)
TGE112628897(CERS3)
RRO104676807(CERS3)
RBB108512618(CERS3)
TFN117070128(CERS3)
PTEH111527358(CERS3)
CJC100406591(CERS3)
SBQ101042956(CERS3)
CSYR103271544(CERS3)
MMUR105871278(CERS3)
LCAT123644504(CERS3)
OGA100941752(CERS3)
MMU545975(Cers3)
MCAL110299355(Cers3)
MPAH110333640(Cers3)
RNO499174(Cers3)
MCOC116102359(Cers3)
MUN110546438(Cers3)
CGE100770521(Cers3)
MAUA101843120(Cers3)
PLEU114695508(Cers3)
MORG121450848(Cers3)
AAMP119802861(Cers3)
NGI103747188(Cers3)
HGL101702770(Cers3)
CPOC100712857(Cers3)
CCAN109700448(Cers3)
DORD105982319(Cers3)
DSP122123985(Cers3)
NCAR124976987
OCU100337851(CERS3)
OPI101520281(CERS3)
TUP102475021(CERS3)
CFA607925(CERS3)
CLUD112653663(CERS3)
VVP112923446(CERS3)
VLG121489824(CERS3)
AML100466254(CERS3)
UMR103659988(CERS3)
UAH113263637(CERS3)
UAR123790831(CERS3)
ELK111152682
LLV125105651
MPUF101687825(CERS3)
ORO101381953(CERS3)
EJU114223819(CERS3)
ZCA113909841(CERS3)
MLX118022993(CERS3)
FCA101099301(CERS3)
PYU121042489(CERS3)
PBG122468302(CERS3)
PTG102968533(CERS3)
PPAD109273347(CERS3)
AJU106972088(CERS3)
HHV120233673(CERS3)
BTA614354(CERS3)
BOM102282323(CERS3)
BIU109575125(CERS3)
BBUB102405478(CERS3)
CHX102169911(CERS3)
OAS101110093(CERS3)
ODA120875266(CERS3)
CCAD122455117(CERS3)
SSC100152265(CERS3)
CFR102505196(CERS3)
CBAI105075152(CERS3)
CDK105093450(CERS3)
VPC102533284(CERS3)
BACU103016103(CERS3)
LVE103082414(CERS3)
OOR101287722(CERS3)
DLE111187344(CERS3)
PCAD102975484(CERS3)
PSIU116749338(CERS3)
ECB100050135(CERS3)
EPZ103561069(CERS3)
EAI106839474(CERS3)
MYB102242969(CERS3)
MYD102759130(CERS3)
MMYO118679202(CERS3)
MLF102428338(CERS3)
MNA107527642(CERS3)
PKL118704309(CERS3)
HAI109380354(CERS3)
DRO112306075(CERS3)
SHON118992022(CERS3)
AJM119049767(CERS3)
PDIC114488632(CERS3)
PHAS123809741(CERS3)
MMF118640928(CERS3)
RFQ117018868(CERS3)
PALE102889613(CERS3)
PGIG120587350(CERS3)
PVP105290275(CERS3)
RAY107515791(CERS3)
MJV108386501(CERS3)
TOD119245188(CERS3)
SARA101550104(CERS3)
LAV100671292(CERS3)
TMU101341813
DNM101414521(CERS3)
MDO100020588(CERS3)
GAS123234193(CERS3)
SHR100919629(CERS3)
PCW110192586(CERS3)
OAA100090484(CERS3)
GGA426648(CERS3)
MGP104912889(CERS3)
CJO107318875(CERS3)
NMEL110403582(CERS3)
APLA101794409(CERS3)
ACYG106036468(CERS3)
AFUL116493542(CERS3)
TGU100223140(CERS3)
LSR110482993(CERS3)
SCAN103816094(CERS3)
PMOA120509089(CERS3)
OTC121343550(CERS3)
PRUF121354481(CERS3)
GFR102036650(CERS3)
FAB101818464(CERS3)
PHI102104999(CERS3)
PMAJ107209495(CERS3)
CCAE111934002(CERS3)
CCW104683905(CERS3)
CBRC103619239(CERS3)
ETL114068286(CERS3)
ZAB102070635(CERS3)
ACHL103806343(CERS3)
FPG101915529(CERS3)
FCH102054381(CERS3)
CLV102090020(CERS3)
EGZ104122870(CERS3)
NNI104017067(CERS3)
PLET104617867(CERS3)
PCRI104028856(CERS3)
ACUN113484146(CERS3)
TALA104368379(CERS3)
PADL103914771(CERS3)
ACHC115341326(CERS3)
HALD104317589(CERS3)
CCRI104163289(CERS3)
CSTI104562225(CERS3)
EHS104516365(CERS3)
CMAC104475655(CERS3)
MUI104541394(CERS3)
FGA104079146(CERS3)
GSTE104257033(CERS3)
LDI104351475(CERS3)
MNB103770554(CERS3)
OHA104339289(CERS3)
AAM106495821(CERS3)
AROW112969803(CERS3)
NPD112948296(CERS3)
DNE112996295(CERS3)
ASN102388749(CERS3)
AMJ102575439(CERS3)
CPOO109309161(CERS3)
GGN109288466(CERS3)
PSS102452581(CERS3)
CMY102939917(CERS3)
CPIC101947682(CERS3)
TST117884533(CERS3)
CABI116816623(CERS3)
MRV120373108(CERS3)
ACS100554835(cers3)
PVT110072978(CERS3)
SUND121934958(CERS3)
PBI103067220(CERS3)
PMUR107283879(CERS3)
PGUT117669869(CERS3)
VKO123030808(CERS3)
PMUA114584561
ZVI118092579(CERS3)
GJA107107422
STOW125424565(CERS3)
XLA379135(cers3.L) 444162(cers3.S)
XTR448139(cers3)
NPR108790075(CERS3)
RTEM120931148(CERS3)
BBUF120985986(CERS3)
BGAR122923564 122927517(CERS3)
DRE100141367(cers3b) 100332380(cers3a)
SRX107719675 107733272 107742325
SANH107662258 107662883 107686553 107692405
SGH107553816 107559674 107562918
CCAR109052074 109087142 109109440
CAUA113051920 113052001 113100321 113105753 113118158
PPRM120464869(cers3b) 120480136(cers3a)
MAMB125254131(cers3b) 125264462(cers3a)
IPU108264168(cers3b) 108271085(cers3a)
PHYP113526340 113536588(cers3)
SMEO124391432(cers3a) 124395980(cers3b)
TFD113649636(cers3b) 113652894(cers3a)
AMEX103027875(cers3) 103029792
EEE113573945(cers3) 113580530
TRU101066117
TNGGSTEN00035593G001
LCO104929719(cers3) 104935212
NCC104957244
CGOB115004452 115009955
ELY117255921(cers3a) 117261833(cers3b)
EFO125887456(cers3b) 125905346(cers3a)
PLEP121944372(cers3a) 121950432(cers3b)
SLUC116045176(cers3a) 116045667(cers3b)
ECRA117947619(cers3a) 117948874(cers3b)
ESP116684187 116694253(cers3) 116702387
PFLV114554884 114560654
GAT120809008(cers3b)
PPUG119196632(cers3b)
MSAM119883380(cers3a) 119901488
CUD121510734(cers3a) 121515377(cers3b)
ALAT119018717(cers3a) 119025088
MZE101477435(cers3) 101480781
ONL100710960 100711452
OAU116313518(cers3b) 116320058(cers3a)
OLA111947541
OML112151761(cers3b)
XMA102234103 102236156
XCO114143780 114160349
XHE116708311 116718874
PRET103462267 103466657
PFOR103133806 103149196(cers3)
PLAI106956231 106959127(cers3)
PMEI106915516 106925757(cers3)
GAF122835013(cers3b) 122846287(cers3a)
CVG107096435 107102400
CTUL119773704(cers3a)
GMU124866838(cers3a) 124877958(cers3b)
NFU107388829
KMR108229836(cers3b) 108250391(cers3a)
ALIM106514312(cers3)
NWH119416282(cers3b) 119429828(cers3a)
AOCE111571929 111581063
MCEP125005721(cers3a) 125014337(cers3b)
CSEM103378927(cers3)
POV109623996 109624784(cers3)
SSEN122772881(cers3a) 122775403(cers3b)
HHIP117759513(cers3a) 117763801(cers3b)
HSP118109828(cers3b) 118112445(cers3a)
LCF108875121 108894327
SDU111220178(cers3) 111228613
SLAL111644372 111653318
XGL120793324(cers3b) 120796400(cers3a)
HCQ109528089
BPEC110162568 110169205
MALB109951998 109967919
BSPL114857840(cers3b)
SASA106562479 106562576
OTW112241699(cers3a)
OMY110506178 110517996
OGO124035401 124035507(cers3a)
ONE115125322
SNH120057070(cers3a)
CCLU121548345 123481019
ELS105014426(cers3)
SFM108941195(cers3)
PKI111845096(cers3)
AANG118215161 118227406(cers3a)
LOC102694534(cers3)
PSPA121295084(cers3a) 121299274
ARUT117427980 117429295
HAZT108664540
BGT106074897
MMER123532589
 » 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.
  Journal
PLoS One 8:e67317 (2013)
DOI:10.1371/journal.pone.0067317
Other DBs
ExplorEnz - The Enzyme Database: 2.3.1.298
IUBMB Enzyme Nomenclature: 2.3.1.298
ExPASy - ENZYME nomenclature database: 2.3.1.298
BRENDA, the Enzyme Database: 2.3.1.298
LinkDB

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