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Details on Person UniProt:Q96G23 CERS2

Class:IdReferenceGeneProduct:149891
_chainChangeLogchain:1-380 added on Sat February 7 2015
_displayNameUniProt:Q96G23 CERS2
_timestamp2026-02-20 21:47:43
chainchain:1-380
checksumC8CEAF6AAD0B4577
commentFUNCTION Ceramide synthase that catalyzes the transfer of the acyl chain from acyl-CoA to a sphingoid base, with high selectivity toward very-long-chain fatty acyl-CoA (chain length C22-C27) (PubMed:17977534, PubMed:18165233, PubMed:18541923, PubMed:19728861, PubMed:20937905, PubMed:22144673, PubMed:22661289, PubMed:26887952, PubMed:29632068). N-acylates sphinganine and sphingosine bases to form dihydroceramides and ceramides in de novo synthesis and salvage pathways, respectively (By similarity) (PubMed:17977534, PubMed:18165233, PubMed:18541923, PubMed:19728861, PubMed:20937905, PubMed:22144673, PubMed:22661289, PubMed:26887952, PubMed:29632068). Plays a non-redundant role in the synthesis of ceramides with very-long-chain fatty acids in kidney, liver and brain. Regulates the abundance of myelin-specific sphingolipids galactosylceramide and sulfatide that affects myelin sheath architecture and motor neuron functions (By similarity).CATALYTIC ACTIVITY a very long-chain fatty acyl-CoA + a sphingoid base = an N-(very-long-chain fatty acyl)-sphingoid base + CoA + H(+)CATALYTIC ACTIVITY docosanoyl-CoA + sphinganine = N-docosanoylsphinganine + CoA + H(+)CATALYTIC ACTIVITY tetracosanoyl-CoA + sphinganine = N-tetracosanoylsphinganine + CoA + H(+)CATALYTIC ACTIVITY hexacosanoyl-CoA + sphinganine = N-hexacosanoylsphinganine + CoA + H(+)CATALYTIC ACTIVITY (15Z)-tetracosenoyl-CoA + sphinganine = N-(15Z-tetracosenoyl)-sphinganine + CoA + H(+)CATALYTIC ACTIVITY 2-hydroxytetracosanoyl-CoA + sphinganine = N-(2-hydroxytetracosanoyl)-sphinganine + CoA + H(+)CATALYTIC ACTIVITY 2-hydroxydocosanoyl-CoA + sphinganine = N-(2-hydroxydocosanoyl)-sphinganine + CoA + H(+)CATALYTIC ACTIVITY 2-hydroxytetracosenoyl-CoA + sphinganine = N-(2-hydroxytetracosenoyl)-sphinganine + CoA + H(+)CATALYTIC ACTIVITY tetracosenoyl-CoA + sphinganine = an N-tetracosenoylsphinganine + CoA + H(+)CATALYTIC ACTIVITY hexacosenoyl-CoA + sphinganine = N-hexacosenoylsphinganine + CoA + H(+)CATALYTIC ACTIVITY tetracosanoyl-CoA + sphing-4-enine = N-tetracosanoyl-sphing-4-enine + CoA + H(+)CATALYTIC ACTIVITY tetracosenoyl-CoA + sphing-4-enine = N-(tetracosenoyl)-sphing-4-enine + CoA + H(+)CATALYTIC ACTIVITY heptadecasphing-4-enine + tetracosanoyl-CoA = N-tetracosanoyl-heptadecasphing-4-enine + CoA + H(+)CATALYTIC ACTIVITY a fatty acyl-CoA + sphing-4-enine = an N-acylsphing-4-enine + CoA + H(+)CATALYTIC ACTIVITY sphing-4-enine + hexadecanoyl-CoA = N-hexadecanoylsphing-4-enine + CoA + H(+)CATALYTIC ACTIVITY sphing-4-enine + octadecanoyl-CoA = N-octadecanoylsphing-4-enine + CoA + H(+)CATALYTIC ACTIVITY eicosanoyl-CoA + sphing-4-enine = N-eicosanoyl-sphing-4-enine + CoA + H(+)CATALYTIC ACTIVITY sphinganine + hexadecanoyl-CoA = N-hexadecanoylsphinganine + CoA + H(+)CATALYTIC ACTIVITY sphinganine + octadecanoyl-CoA = N-(octadecanoyl)-sphinganine + CoA + H(+)CATALYTIC ACTIVITY sphinganine + (9Z)-octadecenoyl-CoA = N-(9Z-octadecenoyl)-sphinganine + CoA + H(+)CATALYTIC ACTIVITY eicosanoyl-CoA + sphinganine = N-eicosanoylsphinganine + CoA + H(+)ACTIVITY REGULATION Ceramide synthase activity is inhibited by sphingosine-1-phosphate.BIOPHYSICOCHEMICAL PROPERTIES Lipid metabolism; sphingolipid metabolism.SUBUNIT Interacts with ATP6V0C, ASGR1, ASGR2 and SLC22A1/OCT1 (PubMed:11543633). Interacts with ELOV1, HSD17B12 and TECR (PubMed:20937905). Interacts with NDUFS2 (By similarity). Interacts with PAQR4; the interaction regulates the stability and activity of CERS2 and is inhibited in presence of ceramides (PubMed:38961186).INTERACTION Expressed in kidney, liver, brain, heart, placenta and lung.DOMAIN The last loop motif confers selectivity toward behenoyl-CoA (docosanoyl-CoA; C22:0-CoA) and lignoceroyl-CoA (tetracosanoyl-CoA; C24:0-CoA) as acyl donors.DOMAIN The predicted Homeobox domain (Homeobox-like region) lacks important residues for DNA-binding. Moreover, the protein localizes to the endoplasmic reticulum membrane, strongly suggesting that it does not constitute a canonical homeobox domain.PTM Acetylated. Deacetylation by SIRT3 increases enzyme activity and promotes mitochondrial ceramide accumulation.PTM Phosphorylated at the C-terminus by CK2, leading to increase the ceramide synthase activity.SIMILARITY Belongs to the sphingosine N-acyltransferase family.SEQUENCE CAUTION Truncated N-terminus.
created[InstanceEdit:143527] Schmidt, EE, 2004-11-12 07:45:10
descriptionrecommendedName: fullName evidence="20"Ceramide synthase 2 shortName evidence="20"CerS2 alternativeName: fullName evidence="19"LAG1 longevity assurance homolog 2 alternativeName: fullName evidence="19"SP260 alternativeName: fullName evidence="22"Sphingosine N-acyltransferase CERS2 ecNumber evidence="13"2.3.1.24 alternativeName: fullName evidence="21"Tumor metastasis-suppressor gene 1 protein alternativeName: fullName evidence="22"Very-long-chain ceramide synthase CERS2 ecNumber evidence="6 8 9 10 12 13 16"2.3.1.297
geneNameCERS2
LASS2
TMSG1
identifierQ96G23
isSequenceChangedFALSE
keywordAcetylation
Endoplasmic reticulum
Glycoprotein
Lipid biosynthesis
Lipid metabolism
Membrane
Phosphoprotein
Proteomics identification
Reference proteome
Transferase
Transmembrane
Transmembrane helix
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
[InstanceEdit:9983091] Weiser, Joel, 2026-02-20
nameCERS2
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8989435] ENSEMBL:ENSG00000143418 CERS2 [Homo sapiens]
secondaryIdentifierCERS2_HUMAN
D3DV06
Q5SZE5
Q9HD96
Q9NW79
sequenceLength380
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:428171] CERS2 [endoplasmic reticulum membrane] [Homo sapiens]
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