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Details on Person UniProt:Q1A3B0-1 Cers3

Class:IdReferenceIsoform:5083154
_chainChangeLogchain:1-383 added on Fri February 6 2015
_displayNameUniProt:Q1A3B0-1 Cers3
_timestamp2026-02-20 21:47:43
chainchain:1-383
checksum98C178A298EBDDEF
commentFUNCTION Ceramide synthase that catalyzes the transfer of the acyl chain from acyl-CoA to a sphingoid base, with high selectivity toward very- and ultra-long-chain fatty acyl-CoA (chain length greater than C22) (PubMed:16753040, PubMed:18541923, PubMed:22038835). N-acylates sphinganine and sphingosine bases to form dihydroceramides and ceramides in de novo synthesis and salvage pathways, respectively (PubMed:16753040, PubMed:18541923, PubMed:22038835). It is crucial for the synthesis of ultra-long-chain ceramides in the epidermis, to maintain epidermal lipid homeostasis and terminal differentiation (PubMed:22038835).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 2-hydroxydocosanoyl-CoA + sphinganine = N-(2-hydroxydocosanoyl)-sphinganine + CoA + H(+)CATALYTIC ACTIVITY 2-hydroxytetracosanoyl-CoA + sphinganine = N-(2-hydroxytetracosanoyl)-sphinganine + CoA + H(+)CATALYTIC ACTIVITY an ultra-long-chain fatty acyl-CoA + a sphingoid base = an N-(ultra-long-chain-acyl)-sphingoid base + CoA + H(+)CATALYTIC ACTIVITY octacosanoyl-CoA + sphinganine = N-(octacosanoyl)-sphinganine + CoA + H(+)CATALYTIC ACTIVITY a fatty acyl-CoA + sphing-4-enine = an N-acylsphing-4-enine + CoA + H(+)CATALYTIC ACTIVITY sphinganine + octadecanoyl-CoA = N-(octadecanoyl)-sphinganine + CoA + H(+)CATALYTIC ACTIVITY 2-hydroxyoctadecanoyl-CoA + sphinganine = N-(2-hydroxyoctadecanoyl)-sphinganine + CoA + H(+)PATHWAY Lipid metabolism; sphingolipid metabolism.SUBCELLULAR LOCATION Predominantly expressed in testis (PubMed:16753040). In skin, present in the upper stratum spinosum and stratum granulosum (at protein level) (PubMed:22038835).DISRUPTION PHENOTYPE Newborn mice die shortly after birth from transepidermal water loss (PubMed:22038835). Defects are due to a complete loss of ultra long chain (more than C26:0) ceramides (PubMed:22038835). Newborn skin appears unwrinkled, erythematous and sticky (PubMed:22038835). Skin is also prone to C.albicans infection (PubMed:22038835).SIMILARITY Belongs to the sphingosine N-acyltransferase family.CAUTION Contains a predicted homeobox domain which is degenerated, lacking residues important for DNA-binding. Moreover, the protein localizes in the endoplasmic reticulum and not in the nucleus, which also argues against homeobox function (PubMed:22038835).
created[InstanceEdit:5083144] Weiser, JD
descriptionrecommendedName: fullName evidence="9"Ceramide synthase 3 shortName evidence="9"CerS3 shortName evidence="9"mCerS3 alternativeName: fullName evidence="10"Dihydroceramide synthase 3 alternativeName: fullName evidence="8"LAG1 longevity assurance homolog 3 alternativeName: fullName evidence="10"Sphingosine N-acyltransferase CERS3 ecNumber evidence="5"2.3.1.24 alternativeName: fullName evidence="10"Ultra-long-chain ceramide synthase CERS3 ecNumber evidence="7"2.3.1.298 alternativeName: fullName evidence="10"Very-long-chain ceramide synthase CERS3 ecNumber evidence="5 6 7"2.3.1.297
geneNameCers3
Lass3
identifierQ1A3B0
isoformParent
isSequenceChangedFALSE
keywordAlternative promoter usage
Endoplasmic reticulum
Lipid biosynthesis
Lipid metabolism
Membrane
Phosphoprotein
Reference proteome
Transferase
Transmembrane
Transmembrane helix
modified[InstanceEdit:5433710] Weiser, JD
[InstanceEdit:5618415] Weiser, JD
[InstanceEdit:5634237] Weiser, JD
[InstanceEdit:5673015] Weiser, JD
[InstanceEdit:6807888] Weiser, JD
[InstanceEdit:9037114] Weiser, JD
[InstanceEdit:9637257] Weiser, JD
[InstanceEdit:9657908] Weiser, JD
[InstanceEdit:9676415] Weiser, JD
[InstanceEdit:9715482] Weiser, JD
[InstanceEdit:9730071] Weiser, JD
[InstanceEdit:9750299] Weiser, JD
[InstanceEdit:9767224] Weiser, Joel
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
[InstanceEdit:9983091] Weiser, Joel, 2026-02-20
nameCers3
referenceDatabase[ReferenceDatabase:2] UniProt
secondaryIdentifierCERS3_MOUSE
E9PVY9
Q195J4
sequenceLength383
species[Species:48892] Mus musculus
variantIdentifierQ1A3B0-1
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