Synonyms |
CerS1, LAG1 longevity assurance homolog 1, Longevity assurance gene 1 protein homolog 1, Protein UOG-1, Sphingoid base N-stearoyltransferase CERS1, EC 2.3.1.299 | LAG1, LASS1, UOG1 |
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:26887952, PubMed:31916624, PubMed:24782409). 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). {ECO:0000250|UniProtKB:P27545, ECO:0000269|PubMed:17977534, ECO:0000269|PubMed:22922758, ECO:0000269|PubMed:23530041, ECO:0000269|PubMed:24782409, ECO:0000269|PubMed:26887952, ECO:0000269|PubMed:31916624}. |