Recombinant Human Very-long-chain enoyl-CoA reductase (TECR) Protein

Function

Involved in both the production of very long-chain fatty acids for sphingolipid synthesis and the degradation of the sphingosine moiety in sphingolipids through the sphingosine 1-phosphate metabolic pathway (PubMed:25049234). Catalyzes the last of the four reactions of the long-chain fatty acids elongation cycle (PubMed:12482854). This endoplasmic reticulum-bound enzymatic process, allows the addition of 2 carbons to the chain of long- and very long-chain fatty acids/VLCFAs per cycle (PubMed:12482854). This enzyme reduces the trans-2,3-enoyl-CoA fatty acid intermediate to an acyl-CoA that can be further elongated by entering a new cycle of elongation (PubMed:12482854). Thereby, it participates in the production of VLCFAs of different chain lengths that are involved in multiple biological processes as precursors of membrane lipids and lipid mediators (PubMed:12482854). Catalyzes the saturation step of the sphingosine 1-phosphate metabolic pathway, the conversion of trans-2-hexadecenoyl-CoA to palmitoyl-CoA (PubMed:25049234)

Subcellular Location

Endoplasmic reticulum membrane (Multi-pass membrane protein)

Disease Association

Intellectual developmental disorder, autosomal recessive 14 (MRT14) : A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptive behavior and manifested during the developmental period. [The disease is caused by variants affecting the gene represented in this entry]

Tissue Specificity

Expressed in most tissues tested. Highly expressed in skeletal muscle

Subunit

Interacts with ELOVL1 and LASS2 (PubMed:20937905). Interacts with HACD1 and HACD2 (via the third lumenal loop), but not with HACD3 and HACD4 (PubMed:38422897). Interacts with ELOVL1, ELOVL2, ELOVL3, ELOVL5 and ELOVL7 in the presence of acyl-CoA; interaction with HACD1/2 and that with ELOVLs are mutually exclusive (PubMed:38422897)

Pathway

Lipid metabolism; sphingolipid metabolism

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