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
HSA: 204219(CERS3)
PTR: 453683(CERS3)
PPS: 100994802(CERS3)
GGO: 101128445(CERS3)
PON: 100451535(CERS3)
NLE: 100590821(CERS3)
MCC: 693696(CERS3)
MCF: 102143963(CERS3)
CSAB: 103231276(CERS3)
CATY: 105593986(CERS3)
PANU: 101007923(CERS3)
RRO: 104676807(CERS3)
RBB: 108512618(CERS3)
TFN: 117070128(CERS3)
PTEH: 111527358(CERS3)
CJC: 100406591(CERS3)
SBQ: 101042956(CERS3)
MMUR: 105871278(CERS3)
MMU: 545975(Cers3)
MCAL: 110299355(Cers3)
MPAH: 110333640(Cers3)
RNO: 499174(Cers3)
MCOC: 116102359(Cers3)
MUN: 110546438(Cers3)
CGE: 100770521(Cers3)
PLEU: 114695508(Cers3)
NGI: 103747188(Cers3)
HGL: 101702770(Cers3)
CCAN: 109700448(Cers3)
OCU: 100337851(CERS3)
TUP: 102475021(CERS3)
CFA: 607925(CERS3)
VVP: 112923446(CERS3)
VLG: 121489824(CERS3)
AML: 100466254(CERS3)
UMR: 103659988(CERS3)
UAH: 113263637(CERS3)
ORO: 101381953(CERS3)
ELK: 111152682
MPUF: 101687825(CERS3)
EJU: 114223819(CERS3)
MLX: 118022993(CERS3)
FCA: 101099301(CERS3)
PYU: 121042489(CERS3)
PTG: 102968533(CERS3)
PPAD: 109273347(CERS3)
AJU: 106972088(CERS3)
HHV: 120233673(CERS3)
BTA: 614354(CERS3)
BOM: 102282323(CERS3)
BIU: 109575125(CERS3)
BBUB: 102405478(CERS3)
CHX: 102169911(CERS3)
OAS: 101110093(CERS3)
ODA: 120875266(CERS3)
CCAD: 122455117(CERS3)
SSC: 100152265(CERS3)
CFR: 102505196(CERS3)
CBAI: 105075152(CERS3)
CDK: 105093450(CERS3)
BACU: 103016103(CERS3)
LVE: 103082414(CERS3)
OOR: 101287722(CERS3)
DLE: 111187344(CERS3)
PCAD: 102975484(CERS3)
ECB: 100050135(CERS3)
EPZ: 103561069(CERS3)
EAI: 106839474(CERS3)
MYB: 102242969(CERS3)
MYD: 102759130(CERS3)
MMYO: 118679202(CERS3)
MNA: 107527642(CERS3)
HAI: 109380354(CERS3)
DRO: 112306075(CERS3)
SHON: 118992022(CERS3)
AJM: 119049767(CERS3)
MMF: 118640928(CERS3)
PALE: 102889613(CERS3)
PGIG: 120587350(CERS3)
RAY: 107515791(CERS3)
MJV: 108386501(CERS3)
TOD: 119245188(CERS3)
LAV: 100671292(CERS3)
TMU: 101341813
MDO: 100020588(CERS3)
SHR: 100919629(CERS3)
PCW: 110192586(CERS3)
OAA: 100090484(CERS3)
GGA: 426648(CERS3)
MGP: 104912889(CERS3)
CJO: 107318875(CERS3)
NMEL: 110403582(CERS3)
APLA: 101794409(CERS3)
ACYG: 106036468(CERS3)
TGU: 100223140(CERS3)
LSR: 110482993(CERS3)
SCAN: 103816094(CERS3)
PMOA: 120509089(CERS3)
GFR: 102036650(CERS3)
FAB: 101818464(CERS3)
PHI: 102104999(CERS3)
PMAJ: 107209495(CERS3)
CCAE: 111934002(CERS3)
CCW: 104683905(CERS3)
ETL: 114068286(CERS3)
FPG: 101915529(CERS3)
FCH: 102054381(CERS3)
CLV: 102090020(CERS3)
EGZ: 104122870(CERS3)
NNI: 104017067(CERS3)
ACUN: 113484146(CERS3)
PADL: 103914771(CERS3)
AAM: 106495821(CERS3)
NPD: 112948296(CERS3)
ASN: 102388749(CERS3)
AMJ: 102575439(CERS3)
CPOO: 109309161(CERS3)
GGN: 109288466(CERS3)
PSS: 102452581(CERS3)
CMY: 102939917(CERS3)
CPIC: 101947682(CERS3)
TST: 117884533(CERS3)
CABI: 116816623(CERS3)
ACS: 100554835(cers3)
PVT: 110072978(CERS3)
PBI: 103067220(CERS3)
PMUR: 107283879(CERS3)
PGUT: 117669869(CERS3)
PMUA: 114584561
ZVI: 118092579(CERS3)
GJA: 107107422
XLA: 379135(cers3.L) 444162(cers3.S)
XTR: 448139(cers3)
NPR: 108790075(CERS3)
DRE: 100141367(cers3b) 100332380(cers3a)
IPU: 108264168 108271085(cers3)
PHYP: 113526340 113536588(cers3)
AMEX: 103027875(cers3) 103029792
EEE: 113573945(cers3) 113580530
TRU: 101066117
LCO: 104929719(cers3) 104935212
NCC: 104957244
ELY: 117255921(cers3a) 117261833(cers3b)
PLEP: 121944372(cers3a) 121950432(cers3b)
SLUC: 116045176(cers3a) 116045667(cers3b)
ECRA: 117947619(cers3a) 117948874(cers3b)
GAT: 120809008(cers3b)
MSAM: 119883380(cers3a) 119901488
CUD: 121510734(cers3a) 121515377(cers3b)
MZE: 101477435(cers3) 101480781
OAU: 116313518(cers3b) 116320058(cers3a)
OLA: 111947541
OML: 112151761(cers3b)
CTUL: 119773704(cers3a)
NFU: 107388829
KMR: 108229836(cers3b) 108250391(cers3a)
ALIM: 106514312(cers3)
CSEM: 103378927(cers3)
POV: 109623996 109624784(cers3)
SDU: 111220178(cers3) 111228613
XGL: 120793324(cers3b) 120796400(cers3a)
HCQ: 109528089
SNH: 120057070(cers3a)
ELS: 105014426(cers3)
SFM: 108941195(cers3)
PKI: 111845096(cers3)
AANG: 118215161 118227406(cers3a)
LOC: 102694534(cers3)
PSPA: 121295084(cers3a) 121299274
HAZT: 108664540
BGT: 106074897
 » 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

DBGET integrated database retrieval system