KEGG   ENZYME: 2.3.1.297
Entry
EC 2.3.1.297                Enzyme                                 

Name
very-long-chain ceramide synthase;
sphingoid base N-very-long-chain fatty acyl-CoA transferase;
mammalian ceramide synthase 2;
CERS3 (gene name);
LASS3 (gene name);
LAG1 (gene name);
LAC1 (gene name);
LOH1 (gene name);
LOH3 (gene name)
Class
Transferases;
Acyltransferases;
Transferring groups other than aminoacyl groups
Sysname
very-long-chain fatty acyl-CoA:sphingoid base N-acyltransferase
Reaction(IUBMB)
a very-long-chain fatty acyl-CoA + a sphingoid base = a very-long-chain ceramide + CoA
Reaction(KEGG)
(other) R01496 R06517 R06527
Substrate
very-long-chain fatty acyl-CoA;
sphingoid base
Product
very-long-chain ceramide;
CoA [CPD:C00010]
Comment
This entry describes ceramide synthase enzymes that are specific for very-long-chain fatty acyl-CoA substrates. The two isoforms from yeast and the plant LOH1 and LOH3 isoforms transfer 24:0 and 26:0 acyl chains preferentially and use phytosphingosine as the preferred sphingoid base. The mammalian CERS2 isoform is specific for acyl donors of 20-26 carbons, which can be saturated or unsaturated. The mammalian CERS3 isoform catalyses this activity, but has a broader substrate range and also catalyses the activity of EC 2.3.1.298, ultra-long-chain ceramide synthase. Both mammalian enzymes can use multiple sphingoid bases, including sphinganine, sphingosine, and phytosphingosine.
History
EC 2.3.1.297 created 2019
Pathway
ec00600  Sphingolipid metabolism
ec01100  Metabolic pathways
Orthology
K04709  very-long-chain ceramide synthase
K24621  very-long-chain ceramide synthase
K24622  very-long-chain ceramide synthase / ultra-long-chain ceramide synthase
Genes
HSA: 204219(CERS3) 29956(CERS2) 79603(CERS4)
PTR: 453683(CERS3) 456435(CERS4) 469482(CERS2)
PPS: 100973710(CERS4) 100973768(CERS2) 100994802(CERS3)
GGO: 101128445(CERS3) 101148109(CERS2)
PON: 100174286(CERS2) 100437726(CERS4) 100451535(CERS3)
NLE: 100579482(CERS4) 100581669(CERS2) 100590821(CERS3)
MCC: 693696(CERS3) 706728(CERS4) 716180(CERS2)
MCF: 101926302(CERS4) 102116814(CERS2) 102143963(CERS3)
CSAB: 103231276(CERS3) 103247210(CERS2)
CATY: 105589123(CERS4) 105590207(CERS2) 105593986(CERS3)
PANU: 101007923(CERS3) 101008992(CERS4) 101026254(CERS2)
RRO: 104676807(CERS3) 104681063(CERS2) 104682288(CERS4)
RBB: 108512618(CERS3) 108533256(CERS2) 108534518(CERS4)
TFN: 117070128(CERS3) 117074717(CERS4) 117075078(CERS2) 117080579 117080580
CJC: 100406591(CERS3) 100412093(CERS2) 100414013(CERS4)
SBQ: 101037762(CERS4) 101042956(CERS3) 101043514(CERS2)
MMUR: 105871278(CERS3) 105872998(CERS2) 105885662(CERS4)
MMU: 545975(Cers3) 67260(Cers4) 76893(Cers2)
MCAL: 110291523(Cers2) 110299355(Cers3) 110299943(Cers4)
MPAH: 110320738(Cers2) 110333640(Cers3) 110336399(Cers4)
RNO: 304208(Cers4) 310667(Cers2) 499174(Cers3)
MCOC: 116068854(Cers4) 116093410(Cers2) 116102359(Cers3)
MUN: 110546438(Cers3) 110560786(Cers2)
CGE: 100758196(Cers4) 100765986(Cers2) 100770521(Cers3)
PLEU: 114682369(Cers2) 114686059(Cers4) 114695508(Cers3)
NGI: 103726528(Cers4) 103730090(Cers2) 103747188(Cers3)
HGL: 101702770(Cers3) 101715336(Cers4) 101716285(Cers2)
CCAN: 109681206(Cers2) 109698441(Cers4) 109700448(Cers3)
OCU: 100337851(CERS3) 100352973(CERS4)
TUP: 102475021(CERS3) 102495526(CERS4) 102502685(CERS2)
CFA: 483187(CERS2) 485008(CERS4) 607925(CERS3)
VVP: 112907770(CERS2) 112909651(CERS4) 112923446(CERS3)
VLG: 121489824(CERS3) 121490849(CERS2) 121495325(CERS4)
AML: 100466254(CERS3) 100475918(CERS4) 100481999(CERS2)
UMR: 103659988(CERS3) 103667533(CERS2) 103681884(CERS4)
UAH: 113242908(CERS4) 113246156(CERS2) 113263637(CERS3)
ORO: 101372787(CERS2) 101379762(CERS4) 101381953(CERS3)
MPUF: 101681709(CERS2) 101687825(CERS3) 101687877(CERS4)
EJU: 114197338(CERS2) 114200818(CERS4) 114223819(CERS3)
MLX: 118000317(CERS2) 118004881(CERS4) 118022993(CERS3)
FCA: 101093918(CERS4) 101094362(CERS2) 101099301(CERS3)
PYU: 121013890(CERS4) 121021024(CERS2) 121042489(CERS3)
PTG: 102968533(CERS3) 102970444(CERS2) 102972870(CERS4)
PPAD: 109254210(CERS4) 109257029(CERS2) 109273347(CERS3)
AJU: 106970780(CERS4) 106972088(CERS3) 106979828(CERS2)
HHV: 120233673(CERS3) 120241292(CERS2) 120244829(CERS4)
BTA: 505233(CERS4) 539223(CERS2) 614354(CERS3)
BOM: 102264858(CERS2) 102275684(CERS4) 102282323(CERS3)
BIU: 109555312(CERS2) 109561659(CERS4) 109575125(CERS3)
BBUB: 102400914(CERS4) 102405478(CERS3) 102409179(CERS2)
CHX: 102169911(CERS3) 102174737(CERS4) 102182556(CERS2)
OAS: 101102559(CERS4) 101109498(CERS2) 101110093(CERS3)
ODA: 120855907(CERS2) 120863889(CERS4) 120875266(CERS3)
CCAD: 122434380(CERS2) 122440335(CERS4) 122455117(CERS3)
SSC: 100127167(CERS4) 100152265(CERS3) 100156737(CERS2)
CFR: 102505196(CERS3) 102511821(CERS2) 102519510(CERS4)
CBAI: 105071736(CERS2) 105075152(CERS3) 105078294(CERS4)
CDK: 105087579(CERS4) 105093450(CERS3) 105103195(CERS2)
BACU: 103009608(CERS2) 103009742(CERS4) 103016103(CERS3)
LVE: 103069846(CERS4) 103076923(CERS2) 103082414(CERS3)
OOR: 101271239(CERS2) 101287722(CERS3) 101289498(CERS4)
DLE: 111165990(CERS4) 111169745(CERS2) 111187344(CERS3)
PCAD: 102975484(CERS3) 102982142(CERS2) 102991761(CERS4)
ECB: 100050135(CERS3) 100055037(CERS2) 100066949(CERS4)
EPZ: 103543133(CERS4) 103547641(CERS2) 103561069(CERS3)
EAI: 106839474(CERS3) 106844495(CERS4) 106846528(CERS2)
MYB: 102242969(CERS3) 102250642(CERS4) 102256999(CERS2)
MYD: 102759130(CERS3) 102767770(CERS4) 102772826(CERS2)
MMYO: 118659187(CERS4) 118672751(CERS2) 118679202(CERS3)
MNA: 107527642(CERS3) 107529900(CERS4) 107539131(CERS2)
HAI: 109372275(CERS2) 109380354(CERS3) 109396095(CERS4)
DRO: 112305258(CERS4) 112306075(CERS3) 112313815(CERS2)
SHON: 118992022(CERS3) 118994065(CERS4)
AJM: 119034748(CERS2) 119049767(CERS3) 119059329(CERS4)
MMF: 118637045(CERS2) 118640928(CERS3) 118643610(CERS4)
PALE: 102885951(CERS2) 102889613(CERS3) 102890907(CERS4)
PGIG: 120585217(CERS2) 120587350(CERS3) 120598783(CERS4)
RAY: 107499037(CERS2) 107515791(CERS3) 107519920(CERS4)
MJV: 108386501(CERS3) 108387090(CERS4) 108409468(CERS2)
TOD: 119237185(CERS2) 119240446(CERS4) 119245188(CERS3)
LAV: 100671292(CERS3) 100673086(CERS2) 100674671(CERS4)
OAA: 100090289(CERS4) 100090484(CERS3) 100091312(CERS2)
GGA: 100859441(CERS2) 420050(CERS4) 426648(CERS3) 428318(CERS4L)
PCOC: 116238425(CERS2) 116240192(CERS4) 116240199 116240888
CJO: 107318875(CERS3) 107324560(CERS2) 107325374 107325633(CERS4)
APLA: 101790332 101790516 101794409(CERS3) 101795233(CERS2)
ACYG: 106036468(CERS3) 106044958 106045005(CERS4) 106049279(CERS2)
TGU: 100217479(CERS4) 100223140(CERS3) 100228761(CERS2)
LSR: 110469450(CERS4) 110476809(CERS2) 110482993(CERS3)
PMOA: 120496355(CERS4) 120502596(CERS2) 120509089(CERS3)
FAB: 101812995(CERS4) 101818464(CERS3)
PMAJ: 107209495(CERS3) 107214659(CERS2) 107215358 107215532
CCAE: 111934002(CERS3) 111939476(CERS2) 111940438(CERS4)
FPG: 101915529(CERS3) 101919852(CERS2) 101922241
FCH: 102047202 102049480(CERS2) 102054381(CERS3)
CLV: 102084875 102090020(CERS3)
EGZ: 104122870(CERS3) 104126317(CERS4) 104129703(CERS2)
NNI: 104014462(CERS2) 104017067(CERS3) 104022916(CERS4)
AAM: 106495414(CERS2) 106495821(CERS3) 106497727(CERS4)
NPD: 112943900(CERS4) 112948296(CERS3) 112952964(CERS2)
ASN: 102368101 102383180(CERS2) 102388749(CERS3)
AMJ: 102575439(CERS3) 102575869(CERS2) 102576734(CERS4)
CPOO: 109309161(CERS3) 109324704(CERS2)
GGN: 109288466(CERS3) 109294738(CERS4)
PSS: 102445918(CERS2) 102447507(CERS4) 102452581(CERS3)
CMY: 102929497(CERS2) 102939917(CERS3)
CPIC: 101932603(CERS2) 101945509 101945790 101947682(CERS3)
CABI: 116816623(CERS3) 116817631(CERS2) 116822588 116825777
ACS: 100554835(cers3) 100565999(cers2)
PVT: 110072464(CERS4) 110072978(CERS3) 110081442(CERS2)
PBI: 103050368 103052910(CERS2) 103067220(CERS3)
PMUR: 107283879(CERS3) 107291598 107292912(CERS2)
TSR: 106544882
PGUT: 117656659 117669869(CERS3) 117670133(CERS2)
PMUA: 114584561 114586234(CERS2) 114588341(CERS4)
ZVI: 118075632(CERS2) 118086384(CERS4) 118092579(CERS3)
XLA: 108699337(cers2.L) 108700334(cers2.S) 379135(cers3.L) 444162(cers3.S)
XTR: 100036728(cers2) 100379773(cers4) 448139(cers3)
NPR: 108789800(CERS2) 108790075(CERS3) 108792262
DRE: 100141367(cers3b) 100330155(cers4b) 100332380(cers3a) 259250(cers4a) 259251(cers2a) 565295(cers2b)
PLEP: 121940075 121944372(cers3a) 121945566(cers2b) 121950432(cers3b) 121952013 121957559(cers2a)
ECRA: 117946735(cers2b) 117947619(cers3a) 117948874(cers3b) 117950656 117953649 117957292(cers2a)
GAT: 120809008(cers3b) 120810545(cers2b) 120816498 120823625 120826405(cers2a)
CUD: 121510734(cers3a) 121512799 121514232(cers2b) 121515377(cers3b) 121519690 121523929(cers2a)
OAU: 116313518(cers3b) 116314136 116319418(cers2a) 116320058(cers3a) 116321135(cers2b) 116324836
OML: 112143751(cers2b) 112147188 112150084 112151761(cers3b) 112162387(cers2a)
CTUL: 119773704(cers3a) 119774094 119779598(cers2a) 119783047(cers2b) 119798456
KMR: 108229836(cers3b) 108230657 108237998(cers2a) 108239552 108241136(cers2b) 108250391(cers3a)
XGL: 120784272(cers2b) 120786098(cers2a) 120790850 120793324(cers3b) 120796400(cers3a) 120799047
LOC: 102694534(cers3) 102698915(cers4) 102699256
LCM: 102356427(CERS2) 102362821(CERS4) 102365974(CERS3)
CMK: 103183569(cers4) 103187682(cers3)
CIN: 100037724(lass6) 100101810(lag1-like3)
BGT: 106074897
GAE: 121382164
SCE: YHL003C(LAG1) YKL008C(LAC1)
KMX: KLMA_30063(LAG1) KLMA_80123(lag1)
NCS: NCAS_0A05070(NCAS0A05070) NCAS_0F00970(NCAS0F00970)
NDI: NDAI_0H01460(NDAI0H01460) NDAI_0K02120(NDAI0K02120)
TPF: TPHA_0C04030(TPHA0C04030) TPHA_0G03260(TPHA0G03260)
TBL: TBLA_0B05480(TBLA0B05480) TBLA_0I00240(TBLA0I00240)
TDL: TDEL_0A02770(TDEL0A02770)
KAF: KAFR_0A01800(KAFR0A01800) KAFR_0D03370(KAFR0D03370)
PIC: PICST_32904(LAG1.1) PICST_41505(LAG1.2) PICST_45900(LAG1.3)
SLB: AWJ20_1430(LAG1) AWJ20_4551(LAG1)
NTE: NEUTE1DRAFT150581(NEUTE1DRAFT_150581) NEUTE1DRAFT81502(NEUTE1DRAFT_81502)
ANG: ANI_1_1620094(An11g00990) ANI_1_194184(An04g00600) ANI_1_2320184(An04g10300)
SPO: SPAC1A6.09c(lag1) SPBC3E7.15c(lac1)
ABP: AGABI1DRAFT106395(AGABI1DRAFT_106395) AGABI1DRAFT113528(AGABI1DRAFT_113528)
ABV: AGABI2DRAFT191824(AGABI2DRAFT_191824)
 » show all
Reference
1  [PMID:11387200]
  Authors
Guillas I, Kirchman PA, Chuard R, Pfefferli M, Jiang JC, Jazwinski SM, Conzelmann A
  Title
C26-CoA-dependent ceramide synthesis of Saccharomyces cerevisiae is operated by Lag1p and Lac1p.
  Journal
EMBO J 20:2655-65 (2001)
DOI:10.1093/emboj/20.11.2655
  Sequence
Reference
2  [PMID:11543633]
  Authors
Pan H, Qin WX, Huo KK, Wan DF, Yu Y, Xu ZG, Hu QD, Gu KT, Zhou XM, Jiang HQ, Zhang PP, Huang Y, Li YY, Gu JR
  Title
Cloning, mapping, and characterization of a human homologue of the yeast longevity assurance gene LAG1.
  Journal
Genomics 77:58-64 (2001)
DOI:10.1006/geno.2001.6614
  Sequence
[hsa:29956]
Reference
3  [PMID:11694577]
  Authors
Schorling S, Vallee B, Barz WP, Riezman H, Oesterhelt D
  Title
Lag1p and Lac1p are essential for the Acyl-CoA-dependent ceramide synthase reaction in Saccharomyces cerevisae.
  Journal
Mol Biol Cell 12:3417-27 (2001)
DOI:10.1091/mbc.12.11.3417
  Sequence
Reference
4  [PMID:15823095]
  Authors
Mizutani Y, Kihara A, Igarashi Y
  Title
Mammalian Lass6 and its related family members regulate synthesis of specific ceramides.
  Journal
Biochem J 390:263-71 (2005)
DOI:10.1042/BJ20050291
  Sequence
[mmu:76893 67260]
Reference
5  [PMID:18165233]
  Authors
Laviad EL, Albee L, Pankova-Kholmyansky I, Epstein S, Park H, Merrill AH Jr, Futerman AH
  Title
Characterization of ceramide synthase 2: tissue distribution, substrate specificity, and inhibition by sphingosine 1-phosphate.
  Journal
J Biol Chem 283:5677-84 (2008)
DOI:10.1074/jbc.M707386200
  Sequence
[hsa:29956] [mmu:76893]
Reference
6  [PMID:19801672]
  Authors
Imgrund S, Hartmann D, Farwanah H, Eckhardt M, Sandhoff R, Degen J, Gieselmann V, Sandhoff K, Willecke K
  Title
Adult ceramide synthase 2 (CERS2)-deficient mice exhibit myelin sheath defects, cerebellar degeneration, and hepatocarcinomas.
  Journal
J Biol Chem 284:33549-60 (2009)
DOI:10.1074/jbc.M109.031971
  Sequence
[mmu:76893]
Other DBs
ExplorEnz - The Enzyme Database: 2.3.1.297
IUBMB Enzyme Nomenclature: 2.3.1.297
ExPASy - ENZYME nomenclature database: 2.3.1.297
BRENDA, the Enzyme Database: 2.3.1.297

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