Ceramide synthase 1 (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)
1_MAAAG 6_ PAAGP 11_ TGPEP 16_ MPSYA 21_ QLVQR 26_ GWGSA 31_ LAAAR 36_ GCTDC 41_ GWGLA 46_ RRGLA 51_ EHAHL 56_ APPEL 61_ LLLAL 66_ GALGW 71_ TALRS 76_ AATAR 81_ LFRPL 86_ AKRCC 91_ LQPRD 96_ AAKMP 101_ ESAWK 106_ FLFYL 111_ GSWSY 116_ SAYLL 121_ FGTDY 126_ PFFHD 131_ PPSVF 136_ YDWTP 141_ GMAVP 146_ RDIAA 151_ AYLLQ 156_ GSFYG 161_ HSIYA 166_ TLYMD 171_ TWRKD 176_ SVVML 181_ LHHVV 186_ TLILI 191_ VSSYA 196_ FRYHN 201_ VGILV 206_ LFLHD 211_ ISDVQ 216_ LEFTK 221_ LNIYF 226_ KSRGG 231_ SYHRL 236_ HALAA 241_ DLGCL 246_ SFGFS 251_ WFWFR 256_ LYWFP 261_ LKVLY 266_ ATSHC 271_ SLRTV 276_ PDIPF 281_ YFFFN 286_ ALLLL 291_ LTLMN 296_ LYWFL 301_ YIVAF 306_ AAKVL 311_ TGQVH 316_ ELKDL 321_ REYDT 326_ AEAQS 331_ LKPSK 336_ AEKPL 341_RNGLV
1: 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)