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
HSA10715(CERS1)
PTR468773(CERS1)
PPS100968130(GDF1) 117974228(CERS1)
GGO109024075(CERS1)
PON100447528(CERS1)
NLE115830373(CERS1)
MCC106994665(CERS1)
MCF107128159(CERS1)
CSAB103234393(CERS1)
CATY105571322(CERS1)
PANU101023016(CERS1)
TGE112613582(CERS1)
RRO104680635(CERS1)
RBB108515632(CERS1)
TFN117074726(CERS1)
PTEH111554297(CERS1)
CJC103789832(CERS1)
SBQ101041201(CERS1)
CSYR103255092(CERS1)
MMUR105855008(CERS1)
LCAT123638527(CERS1)
OGA100965545(CERS1)
MMU93898(Cers1)
MCAL110299631(Cers1)
MPAH110336871(Cers1)
RNO290658(Cers1)
MCOC116069461(Cers1)
MUN110549168(Cers1)
CGE100765511(Cers1)
MAUA101834237(Cers1)
PLEU114682854(Cers1)
MORG121462603(Cers1)
AAMP119811106(Cers1)
NGI103726856(Cers1)
HGL101705418(Cers1)
CPOC100720321(Cers1)
CCAN109684934(Cers1)
DORD105994765(Cers1)
DSP122100835(Cers1)
NCAR124969610
OPI101528668(CERS1)
TUP102475765(CERS1)
CFA609789(CERS1)
CLUD112666914(CERS1)
VVP112913073(CERS1)
VLG121494456(CERS1)
AML100478537(CERS1)
UMR103675149(CERS1)
UAH113256735(CERS1)
UAR123779847(CERS1)
ELK111144244
LLV125081927
MPUF101684554(CERS1)
ORO101380694(CERS1)
EJU114196502(CERS1)
ZCA113917394(CERS1)
MLX118005654(CERS1)
FCA101089084(CERS1)
PYU121011039(CERS1)
PBG122487126(CERS1)
PTG102961418(CERS1)
PPAD109247644(CERS1)
AJU106969135(CERS1)
HHV120229350(CERS1)
BTA615296(CERS1)
BOM102269743(CERS1)
BIU109560813(CERS1)
BBUB102401086(CERS1)
CHX102189571(CERS1)
OAS101107095(CERS1)
ODA120864284(CERS1)
CCAD122440773(CERS1)
SSC100525450(CERS1)
CFR102507344(CERS1)
CBAI105082588(CERS1)
CDK105101886(CERS1)
VPC102531422(CERS1)
BACU103003098(CERS1)
LVE103091398(CERS1)
OOR101273399(CERS1)
DLE111166519(CERS1)
PCAD102977839(CERS1)
PSIU116750406(CERS1)
ECB100069594(CERS1)
EAI106846409(CERS1)
MYB102249252(CERS1)
MYD102769776(CERS1)
MMYO118657665(CERS1)
MNA107535343(CERS1)
PKL118726060(CERS1)
HAI109382399(CERS1)
DRO112305215(CERS1)
SHON118975143(CERS1)
AJM119046855(CERS1)
PDIC114503205(CERS1)
PHAS123828516(CERS1)
MMF118615483(CERS1)
RFQ117037537(CERS1)
PALE102885878(CERS1)
PGIG120606575(CERS1)
PVP105296670(CERS1)
RAY107503723(CERS1)
MJV108406026(CERS1)
TOD119241139(CERS1)
SARA101546837(CERS1)
LAV100667031(CERS1)
TMU101347699
MDO100016686(CERS1)
SHR100934844(CERS1)
PCW110223377(CERS1)
OAA100078502(CERS1)
GGA420118(CERS1)
PCOC116235734(CERS1)
MGP100547857(CERS1)
CJO107325354(CERS1)
NMEL110388870(CERS1)
APLA101804779(CERS1)
ACYG106047960(CERS1)
AFUL116499027(CERS1)
TGU100230030(CERS1)
LSR110478333(CERS1)
SCAN108963610(CERS1)
PMOA120499066(CERS1)
OTC121346963(CERS1)
PRUF121352690(CERS1)
GFR102035945(CERS1)
PHI102107684(CERS1)
CCW104697388(CERS1)
CBRC103625546(CERS1)
ETL114071345(CERS1)
ACHL103807133(CERS1)
FPG101923637(CERS1)
FCH102046386(CERS1)
CLV102098532(CERS1)
EGZ104129784(CERS1)
NNI104013688(CERS1)
PLET104617837(CERS1)
PCRI104027453(CERS1)
ACUN113489365(CERS1)
TALA104365913(CERS1)
PADL103923274(CERS1)
ACHC115349598(CERS1)
HALD104324485(CERS1)
CCRI104157429(CERS1)
EHS104510525(CERS1)
MUI104534905(CERS1)
FGA104075258(CERS1)
GSTE104260787(CERS1)
LDI104353917(CERS1)
MNB103780032(CERS1)
OHA104327354(CERS1)
AROW112965583(CERS1)
NPD112946884(CERS1)
DNE112992321(CERS1)
ASN102384359(CERS1)
AMJ102563913(CERS1)
CPOO109312345(CERS1)
GGN109294983(CERS1)
CMY102935204(CERS1)
CPIC103305686(CERS1)
TST117869035(CERS1)
CABI116826209(CERS1)
MRV120391554(CERS1)
PVT110072532(CERS1)
PBI103067620(CERS1)
PMUR107282886(CERS1)
TSR106543865(CERS1)
PGUT117668600(CERS1)
VKO123036639(CERS1)
PMUA114588648(CERS1)
ZVI118086917(CERS1)
GJA107105635(CERS1)
STOW125433276(CERS1)
XLA108703695(cers1.S) 108713514(cers1.L)
XTR100485129(cers1)
NPR108796582(CERS1)
RTEM120909208(CERS1)
BBUF120991588(CERS1)
BGAR122931412(CERS1)
DRE568169(cers1)
SRX107727553(cers1)
SANH107698599 107702637
SGH107549387(cers1) 107566364
CCAR109073214
CAUA113039432 113040275 113100970
PPRM120486384(cers1)
MAMB125263338(cers1)
IPU100528482(cers1)
PHYP113536501(cers1)
SMEO124391046(cers1)
TFD113650366(cers1)
AMEX103043497(cers1)
EEE113577802(cers1)
TRU101079773(cers1)
TNGGSTEN00028872G001
LCO104918072(cers1)
ELY117253847(cers1)
EFO125895282(cers1)
PLEP121966717(cers1)
SLUC116055207 116055250(cers1)
ECRA117951104(cers1)
ESP116695100(cers1)
PFLV114562014(cers1)
GAT120824010(cers1)
PPUG119216213(cers1)
MSAM119900698(cers1)
CUD121511871(cers1)
ALAT119028202(cers1)
MZE101468648(cers1)
ONL100703783(cers1)
OAU116324952(cers1)
OLA101166466(cers1)
OML112150796(cers1)
XMA102238205(cers1)
XCO114150769(cers1)
XHE116725441(cers1)
PRET103464102(cers1)
PFOR103138509(cers1)
PLAI106950667(cers1)
PMEI106933767(cers1)
GAF122838625(cers1)
CVG107101216(cers1)
CTUL119785797(cers1)
GMU124873561(cers1)
NFU107393118(cers1)
KMR108230660(cers1)
ALIM106511910(cers1)
NWH119407819(cers1)
AOCE111571325(cers1)
MCEP125009404(cers1)
CSEM103393467(cers1)
POV109625614(cers1)
SSEN122781273(cers1)
HHIP117759736(cers1)
HSP118120680(cers1)
LCF108881342 108888330
SDU111216491(cers1)
SLAL111650208(cers1)
XGL120791315(cers1)
HCQ109516892(cers1)
BPEC110160186 110160210(cers1)
MALB109967431(cers1)
BSPL114853210(cers1)
SASA106573829 106613669
OTW112250774 112252178
OMY110499356 110524188
OGO123998116 123998665
ONE115108216(cers1) 115109189
SALP111952102 111976086
SNH120054066 120054711
CCLU121533626 121576828
ELS105011446(cers1)
SFM108919894 108921389
PKI111847689 111860256
AANG118224554(cers1) 118230622
LOC102695945(cers1)
PSPA121300765 121301527
ARUT117428976 117966306
LCM102351990(CERS1)
CMK103181964(cers1)
BFO118411284
BBEL109468792
CIN100177090 100184115 100186491
SCLV120339784
SPU592336
APLC110975400 110984193
SKO100378290 100378441 102803080 102803205
BCOO119080479
ISCIscW_ISCW007816
DSV119456326
RSAN119393113
RMP119171909
VDE111247331 111248462
VJA111264486 111267351
TUT107366570
DPTE113789827 113791308 113796244 113798399
DFR124493629 124493675 124494961 124496425 124496491 124496761 124498736
CSCU111612809
PTEP107453109 107455157 107457454
SDM118197250
CELCELE_Y6B3B.10(lagr-1)
CBRCBG_13635(Cbr-lagr-1)
NAINECAME_08564
HROHELRODRAFT_77285
GAE121392529
LAK106174562
SMMSmp_122050
OVIT265_05312 T265_08104
EGLEGR_06452 EGR_07977
NVE5522174
EPA110240089
ATEN116300026
ADF107340796
AMIL114966382
PDAM113670811 113670839
SPIS111324309 111324334
DGT114522828
XEN124451328
HMG100198819 100215789
TADTRIADDRAFT_52373 TRIADDRAFT_58575
AQU100641959 109585638
ACANACA1_190040 ACA1_208380
 » 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
LinkDB

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