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Details on Person UniProt:P27544 CERS1
| Class:Id | ReferenceGeneProduct:58743 |
|---|---|
| _chainChangeLog | initiator methionine:1 added on Fri February 6 2015;chain:2-350 added on Fri February 6 2015;initiator methionine:1 for 58743 removed on Fri Nov 03 2023;initiator methionine: for 58743 added on Fri Nov 03 2023;initiator methionine: for 58743 removed on Fri Aug 15 2025;initiator methionine:1 for 58743 added on Fri Aug 15 2025 |
| _displayName | UniProt:P27544 CERS1 |
| _timestamp | 2026-02-20 21:47:42 |
| chain | initiator methionine:1 chain:2-350 |
| checksum | F102C12C47DB4162 |
| comment | FUNCTION Ceramide synthase that catalyzes the transfer of the acyl chain from acyl-CoA to a sphingoid base, with high selectivity toward stearoyl-CoA (octadecanoyl-CoA; C18:0-CoA) (PubMed:17977534, PubMed:23530041, PubMed:26887952, PubMed:31916624). N-acylates sphinganine and sphingosine bases to form dihydroceramides and ceramides in de novo synthesis and salvage pathways, respectively (PubMed:17977534, PubMed:23530041, PubMed:24782409, PubMed:26887952, PubMed:31916624). Plays a predominant role in skeletal muscle in regulating C18 ceramide and dihydroceramide levels with an impact on whole-body glucose metabolism and insulin sensitivity. Protects from diet-induced obesity by suppressing the uptake of glucose in multiple organs in a FGF21-dependent way (By similarity). Generates C18 ceramides in the brain, playing a critical role in cerebellar development and Purkinje cell function (By similarity). In response to cellular stress mediates mitophagy, a known defense mechanism against cell transformation and aging. Upon mitochondria fission, generates C18 ceramides that anchor lipidated MAP1LC3B/LC3B-II autophagolysosomes to outer mitochondrial membranes to eliminate damaged mitochondria (PubMed:22922758).CATALYTIC ACTIVITY a sphingoid base + octadecanoyl-CoA = an N-octadecanoyl-sphingoid base + CoA + H(+)CATALYTIC ACTIVITY sphinganine + octadecanoyl-CoA = N-(octadecanoyl)-sphinganine + CoA + H(+)CATALYTIC ACTIVITY hexadecasphinganine + octadecanoyl-CoA = N-octadecanoylhexadecasphinganine + CoA + H(+)CATALYTIC ACTIVITY sphing-4-enine + octadecanoyl-CoA = N-octadecanoylsphing-4-enine + CoA + H(+)CATALYTIC ACTIVITY heptadecasphing-4-enine + octadecanoyl-CoA = N-octadecanoyl-heptadecasphing-4-enine + CoA + H(+)CATALYTIC ACTIVITY 2-hydroxyoctadecanoyl-CoA + sphinganine = N-(2-hydroxyoctadecanoyl)-sphinganine + CoA + H(+)CATALYTIC ACTIVITY eicosanoyl-CoA + sphinganine = N-eicosanoylsphinganine + CoA + H(+)ACTIVITY REGULATION Inhibited by fumonisin B1.PATHWAY Lipid metabolism; sphingolipid metabolism.SUBCELLULAR LOCATION Acetylated. Deacetylation by SIRT3 increases enzyme activity and promotes mitochondrial ceramide accumulation.DISEASE The disease is caused by variants affecting the gene represented in this entry.MISCELLANEOUS This protein is produced by a bicistronic gene which also produces the GDF1 protein from a non-overlapping reading frame.SIMILARITY Belongs to the sphingosine N-acyltransferase family. |
| description | recommendedName: fullName evidence="13"Ceramide synthase 1 shortName evidence="13"CerS1 alternativeName: fullName evidence="15"LAG1 longevity assurance homolog 1 alternativeName: fullName evidence="1"Longevity assurance gene 1 protein homolog 1 alternativeName: fullName evidence="14"Protein UOG-1 alternativeName: fullName evidence="16"Sphingoid base N-stearoyltransferase CERS1 ecNumber evidence="4 7 9 10"2.3.1.299 |
| geneName | CERS1 LAG1 LASS1 UOG1 |
| identifier | P27544 |
| isSequenceChanged | FALSE |
| keyword | Acetylation Alternative splicing Disease variant Endoplasmic reticulum Epilepsy Lipid biosynthesis Lipid metabolism Membrane Neurodegeneration Proteomics identification Reference proteome Transferase Transmembrane Transmembrane helix |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [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 |
| name | CERS1 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8998802] ENSEMBL:ENSG00000223802 CERS1 [Homo sapiens] |
| secondaryIdentifier | CERS1_HUMAN |
| sequenceLength | 350 |
| species | [Species:48887] Homo sapiens |
| (isoformParent) | [ReferenceIsoform:149890] UniProt:P27544-2 CERS1 [Homo sapiens] [ReferenceIsoform:403496] UniProt:P27544-1 CERS1 [Homo sapiens] |
| (referenceEntity) | [EntityWithAccessionedSequence:428131] CERS1 [endoplasmic reticulum membrane] [Homo sapiens] |
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No pathways have been reviewed or authored by UniProt:P27544 CERS1 (58743)
