Recombinant Human Ceramide synthase 1 (CERS1), Truncated

Uniprot Id

P27544

Target Species

Human

Target Name

CERS1

Target Full Name

Ceramide synthase 1

Target 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). N-acylates sphinganine and sphingosine bases to form dihydroceramides and ceramides in de novo synthesis and salvage pathways, respectively. 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. Generates C18 ceramides in the brain, playing a critical role in cerebellar development and Purkinje cell function. 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.

Target Involvement

Epilepsy, progressive myoclonic 8 (EPM8)

Target Subcellular Location

Endoplasmic reticulum membrane; Multi-pass membrane protein.

Target Synonyms

CERS1; LAG1; LASS1; UOG1; Ceramide synthase 1; CerS1; LAG1 longevity assurance homolog 1; Longevity assurance gene 1 protein homolog 1; Protein UOG-1; Sphingoid base N-stearoyltransferase CERS1