Summary

Name MECR
Full Name Enoyl-[acyl-carrier-protein] reductase, mitochondrial
Synonyms EC 1.3.1.104, 2-enoyl thioester reductase, Nuclear receptor-binding factor 1, HsNrbf-1, NRBF-1 | NBRF1
Primary ID Q9BV79
Links - -
Type protein
Relations 4
Pathways Fatty Acid Beta-Oxidation
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 (PubMed:12654921, PubMed:18479707, PubMed:27817865). May provide the octanoyl chain used for lipoic acid biosynthesis, regulating protein lipoylation and mitochondrial respiratory activity particularly in Purkinje cells (By similarity). Involved in iron homeostasis; affecting Fe-S cluster assembly and ceramide metabolism (PubMed:37653044). Required for proper morphology and bioenergetic functions of mitochondria (PubMed:37653044). Required for maintenance of neurons (By similarity). {ECO:0000250|UniProtKB:Q9DCS3, ECO:0000250|UniProtKB:Q9V6U9, ECO:0000269|PubMed:12654921, ECO:0000269|PubMed:18479707, ECO:0000269|PubMed:27817865, ECO:0000269|PubMed:37653044}.

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Relations

Regulator Mechanism target score
+ down-regulates quantity img/direct_inhibition.png chemical modification trans-dec-2-enoyl-CoA 0.8
Publications: 1 Organism: Homo Sapiens
+ down-regulates quantity img/direct_inhibition.png chemical modification NADPH(4-) 0.8
Publications: 1 Organism: Homo Sapiens
+ up-regulates quantity img/direct-activation.png chemical modification decanoyl-CoA 0.8
Publications: 1 Organism: Homo Sapiens
+ up-regulates quantity img/direct-activation.png chemical modification NADP(3-) 0.8
Publications: 1 Organism: Homo Sapiens