ELOVL fatty acid elongase 1 (ELOVL1)

ELOVL fatty acid elongase 1 (ELOVL1) is a membrane-bound enzyme located in the endoplasmic reticulum that catalyzes the first and rate-limiting step of the fatty acid elongation cycle, specifically facilitating the addition of two carbon units to long-chain and very long-chain saturated and monounsaturated fatty acyl-CoA substrates via a condensation reaction with malonyl-CoA[1][2][3]. ELOVL1 is fundamental for the biosynthesis of very long chain fatty acids (VLCFAs), which are essential precursors for membrane lipids and sphingolipids critical for myelin sheath integrity, skin barrier function, and other biological roles[1][2][3]. Mutations in ELOVL1 are associated with rare neurological and dermatological syndromes involving hypomyelination, ichthyosis, and spasticity, indicating its vital importance in human physiology[1][2].

Other names

ELOVL1Elongation of very long chain fatty acids protein 1

Inhibition of fatty acid chain elongation (for enzyme inhibitors, experimental)[3]