| Tissue Specificity | Highly expressed in skeletal and heart muscle. Expressed at lower level in placenta, liver, kidney and pancreas. Weakly or not expressed in lung. |
| Function | Catalyzes the NADPH-dependent reduction of trans-2-enoyl thioesters in mitochondrial fatty acid synthesis (fatty acid synthesis type II). Fatty acid chain elongation in mitochondria uses acyl carrier protein (ACP) as an acyl group carrier, but the enzyme accepts both ACP and CoA thioesters as substrates in vitro. Displays a preference for medium-chain over short- and long-chain substrates. May provide the octanoyl chain used for lipoic acid biosynthesis, regulating protein lipoylation and mitochondrial respiratory activity particularly in Purkinje cells. Involved in iron homeostasis.affecting Fe-S cluster assembly and ceramide metabolism. Required for proper morphology and bioenergetic functions of mitochondria. Required for maintenance of neurons. |
| Protein Name | Enoyl-Acyl-Carrier-Protein Reductase - Mitochondrial2-Enoyl Thioester ReductaseNuclear Receptor-Binding Factor 1Hsnrbf-1Nrbf-1 |
| Database Links | Reactome: R-HSA-77346 |
| Cellular Localisation | Isoform 1: MitochondrionIsoform 2: CytoplasmNucleus |
| Alternative Protein Names | Enoyl-Acyl-Carrier-Protein Reductase - Mitochondrial protein2-Enoyl Thioester Reductase proteinNuclear Receptor-Binding Factor 1 proteinHsnrbf-1 proteinNrbf-1 proteinMECR proteinNBRF1 proteinCGI-63 protein |
Information sourced from Uniprot.org
