Trans-2,3-enoyl-CoA reductase (TECR)

Trans-2,3-enoyl-CoA reductase (TECR) is an endoplasmic reticulum-associated enzyme encoded by the TECR gene in humans, belonging to the steroid 5-alpha reductase family. It catalyzes the final (saturation) step in microsomal long- and very long-chain fatty acid (VLCFA) elongation by reducing trans-2,3-enoyl-CoA intermediates to their saturated acyl-CoA forms using NADPH as a cofactor. TECR plays a central role in the production of saturated and monounsaturated VLCFAs, which are major constituents of sphingolipids, and participates in both lipid biosynthesis and degradation pathways, including an essential step in sphingosine degradation via the sphingosine-1-phosphate metabolic pathway. Mutations in TECR are associated with non-syndromic mental retardation, likely through disruption of neural lipid metabolism.

Other names

TECRTERtrans-2-enoyl-CoA reductaseNADPH-dependent trans-2-enoyl-CoA reductaseacyl-CoA:NADP+ trans-2-oxidoreductaseGPSN2SC2MRT14

Catalyzes reduction of trans-2,3-enoyl-CoA to saturated acyl-CoA in fatty acid elongation; uses NADPH as electron donor; key step in very long-chain fatty acid and sphingolipid synthesis