KEGG   ENZYME: 2.3.1.299
Entry
EC 2.3.1.299                Enzyme                                 

Name
sphingoid base N-stearoyltransferase;
mammalian ceramide synthase 1;
LASS1 (gene name);
UOG1 (gene name);
CERS1 (gene name)
Class
Transferases;
Acyltransferases;
Transferring groups other than aminoacyl groups
Sysname
stearoyl-CoA:sphingoid base N-stearoyltransferase
Reaction(IUBMB)
stearoyl-CoA + a sphingoid base = an N-(stearoyl)-sphingoid base + CoA
Reaction(KEGG)
(other) R01496 R06517
Substrate
stearoyl-CoA [CPD:C00412];
sphingoid base
Product
N-(stearoyl)-sphingoid base;
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 1 (CERS1) is structurally and functionally distinctive from all other CERS enzymes, and is specific for stearoyl-CoA as the acyl donor. It can use multiple sphingoid bases including sphinganine, sphingosine, and phytosphingosine.
History
EC 2.3.1.299 created 2019
Pathway
ec00600  Sphingolipid metabolism
ec01100  Metabolic pathways
Orthology
K04710  sphingoid base N-stearoyltransferase
Genes
HSA: 10715(CERS1)
PTR: 468773(CERS1)
PPS: 100968130(GDF1) 117974228(CERS1)
GGO: 109024075(CERS1)
PON: 100447528(CERS1)
NLE: 115830373(CERS1)
MCC: 106994665(CERS1)
MCF: 107128159(CERS1)
CSAB: 103234393(CERS1)
CATY: 105571322(CERS1)
PANU: 101023016(CERS1)
RRO: 104680635(CERS1)
RBB: 108515632(CERS1)
TFN: 117074726(CERS1)
PTEH: 111554297(CERS1)
CJC: 103789832(CERS1)
SBQ: 101041201(CERS1)
MMUR: 105855008(CERS1)
MMU: 93898(Cers1)
MCAL: 110299631(Cers1)
MPAH: 110336871(Cers1)
RNO: 290658(Cers1)
MCOC: 116069461(Cers1)
MUN: 110549168(Cers1)
CGE: 100765511(Cers1)
PLEU: 114682854(Cers1)
NGI: 103726856(Cers1)
HGL: 101705418(Cers1)
CPOC: 100720321(Cers1)
CCAN: 109684934(Cers1)
OPI: 101528668(CERS1)
TUP: 102475765(CERS1)
CFA: 609789(CERS1)
VVP: 112913073(CERS1)
VLG: 121494456(CERS1)
AML: 100478537(CERS1)
UMR: 103675149(CERS1)
UAH: 113256735(CERS1)
ORO: 101380694(CERS1)
ELK: 111144244
MPUF: 101684554(CERS1)
EJU: 114196502(CERS1)
MLX: 118005654(CERS1)
FCA: 101089084(CERS1)
PYU: 121011039(CERS1)
PBG: 122487126(CERS1)
PTG: 102961418(CERS1)
PPAD: 109247644(CERS1)
AJU: 106969135(CERS1)
HHV: 120229350(CERS1)
BTA: 615296(CERS1)
BOM: 102269743(CERS1)
BIU: 109560813(CERS1)
BBUB: 102401086(CERS1)
CHX: 102189571(CERS1)
OAS: 101107095(CERS1)
ODA: 120864284(CERS1)
CCAD: 122440773(CERS1)
SSC: 100525450(CERS1)
CFR: 102507344(CERS1)
CBAI: 105082588(CERS1)
CDK: 105101886(CERS1)
BACU: 103003098(CERS1)
LVE: 103091398(CERS1)
OOR: 101273399(CERS1)
DLE: 111166519(CERS1)
PCAD: 102977839(CERS1)
PSIU: 116750406(CERS1)
ECB: 100069594(CERS1)
EAI: 106846409(CERS1)
MYB: 102249252(CERS1)
MYD: 102769776(CERS1)
MMYO: 118657665(CERS1)
MNA: 107535343(CERS1)
PKL: 118726060(CERS1)
HAI: 109382399(CERS1)
DRO: 112305215(CERS1)
SHON: 118975143(CERS1)
AJM: 119046855(CERS1)
PDIC: 114503205(CERS1)
MMF: 118615483(CERS1)
RFQ: 117037537(CERS1)
PALE: 102885878(CERS1)
PGIG: 120606575(CERS1)
RAY: 107503723(CERS1)
MJV: 108406026(CERS1)
TOD: 119241139(CERS1)
LAV: 100667031(CERS1)
TMU: 101347699
MDO: 100016686(CERS1)
SHR: 100934844(CERS1)
PCW: 110223377(CERS1)
OAA: 100078502(CERS1)
GGA: 420118(CERS1)
PCOC: 116235734(CERS1)
MGP: 100547857(CERS1)
CJO: 107325354(CERS1)
NMEL: 110388870(CERS1)
APLA: 101804779(CERS1)
ACYG: 106047960(CERS1)
TGU: 100230030(CERS1)
LSR: 110478333(CERS1)
SCAN: 108963610(CERS1)
PMOA: 120499066(CERS1)
OTC: 121346963(CERS1)
PRUF: 121352690(CERS1)
GFR: 102035945(CERS1)
PHI: 102107684(CERS1)
CCW: 104697388(CERS1)
ETL: 114071345(CERS1)
FPG: 101923637(CERS1)
FCH: 102046386(CERS1)
CLV: 102098532(CERS1)
EGZ: 104129784(CERS1)
NNI: 104013688(CERS1)
ACUN: 113489365(CERS1)
PADL: 103923274(CERS1)
AROW: 112965583(CERS1)
NPD: 112946884(CERS1)
DNE: 112992321(CERS1)
ASN: 102384359(CERS1)
AMJ: 102563913(CERS1)
CPOO: 109312345(CERS1)
GGN: 109294983(CERS1)
CMY: 102935204(CERS1)
CPIC: 103305686(CERS1)
TST: 117869035(CERS1)
CABI: 116826209(CERS1)
MRV: 120391554(CERS1)
PVT: 110072532(CERS1)
PBI: 103067620(CERS1)
PMUR: 107282886(CERS1)
TSR: 106543865(CERS1)
PGUT: 117668600(CERS1)
VKO: 123036639(CERS1)
PMUA: 114588648(CERS1)
ZVI: 118086917(CERS1)
GJA: 107105635(CERS1)
XLA: 108703695(cers1.S) 108713514(cers1.L)
XTR: 100485129(cers1)
NPR: 108796582(CERS1)
DRE: 568169(cers1)
SRX: 107727553(cers1)
SGH: 107549387(cers1) 107566364
CCAR: 109073214
IPU: 100528482(cers1)
PHYP: 113536501(cers1)
AMEX: 103043497(cers1)
EEE: 113577802(cers1)
TRU: 101079773(cers1)
LCO: 104918072(cers1)
ELY: 117253847(cers1)
PLEP: 121966717(cers1)
SLUC: 116055207 116055250(cers1)
ECRA: 117951104(cers1)
PFLV: 114562014(cers1)
GAT: 120824010(cers1)
PPUG: 119216213(cers1)
MSAM: 119900698(cers1)
CUD: 121511871(cers1)
MZE: 101468648(cers1)
ONL: 100703783(cers1)
OAU: 116324952(cers1)
OLA: 101166466(cers1)
OML: 112150796(cers1)
XMA: 102238205(cers1)
XCO: 114150769(cers1)
XHE: 116725441(cers1)
PRET: 103464102(cers1)
GAF: 122838625(cers1)
CVG: 107101216(cers1)
CTUL: 119785797(cers1)
NFU: 107393118(cers1)
KMR: 108230660(cers1)
ALIM: 106511910(cers1)
AOCE: 111571325(cers1)
CSEM: 103393467(cers1)
POV: 109625614(cers1)
SSEN: 122781273(cers1)
HHIP: 117759736(cers1)
SDU: 111216491(cers1)
SLAL: 111650208(cers1)
XGL: 120791315(cers1)
HCQ: 109516892(cers1)
BPEC: 110160186 110160210(cers1)
MALB: 109967431(cers1)
ELS: 105011446(cers1)
AANG: 118224554(cers1) 118230622
LOC: 102695945(cers1)
LCM: 102351990(CERS1)
CMK: 103181964(cers1)
BFO: 118411284
BBEL: 109468792
SCLV: 120339784
SPU: 592336
DSV: 119456326
RSAN: 119393113
RMP: 119171909
TUT: 107366570
CSCU: 111612809
SDM: 118197250
CEL: CELE_Y6B3B.10(lagr-1)
CBR: CBG_13635(Cbr-lagr-1)
GAE: 121392529
LAK: 106174562
NVE: 5522174
EPA: 110240089
ATEN: 116300026
ADF: 107340796
AMIL: 114966382
DGT: 114522828
 » show all
Reference
1  [PMID:12105227]
  Authors
Venkataraman K, Riebeling C, Bodennec J, Riezman H, Allegood JC, Sullards MC, Merrill AH Jr, Futerman AH
  Title
Upstream of growth and differentiation factor 1 (uog1), a mammalian homolog of the yeast longevity assurance gene 1 (LAG1), regulates N-stearoyl-sphinganine (C18-(dihydro)ceramide) synthesis in a fumonisin B1-independent manner in mammalian cells.
  Journal
J Biol Chem 277:35642-9 (2002)
DOI:10.1074/jbc.M205211200
  Sequence
[hsa:10715] [mmu:93898]
Reference
2  [PMID:22661289]
  Authors
Kim HJ, Qiao Q, Toop HD, Morris JC, Don AS
  Title
A fluorescent assay for ceramide synthase activity.
  Journal
J Lipid Res 53:1701-7 (2012)
DOI:10.1194/jlr.D025627
Reference
3  [PMID:29262618]
  Authors
Wang Z, Wen L, Zhu F, Wang Y, Xie Q, Chen Z, Li Y
  Title
Overexpression of ceramide synthase 1 increases C18-ceramide and leads to lethal  autophagy in human glioma.
  Journal
Oncotarget 8:104022-104036 (2017)
DOI:10.18632/oncotarget.21955
Reference
4  [PMID:30605666]
  Authors
Turpin-Nolan SM, Hammerschmidt P, Chen W, Jais A, Timper K, Awazawa M, Brodesser S, Bruning JC
  Title
CerS1-Derived C18:0 Ceramide in Skeletal Muscle Promotes Obesity-Induced Insulin  Resistance.
  Journal
Cell Rep 26:1-10.e7 (2019)
DOI:10.1016/j.celrep.2018.12.031
Other DBs
ExplorEnz - The Enzyme Database: 2.3.1.299
IUBMB Enzyme Nomenclature: 2.3.1.299
ExPASy - ENZYME nomenclature database: 2.3.1.299
BRENDA, the Enzyme Database: 2.3.1.299

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