Target intelligence / Profile preview

Mitochondrial trans-2-enoyl-CoA reductase (MECR)

Target
MECR
Molecular classification
Enzyme, Zinc-containing alcohol dehydrogenase family, Quinone oxidoreductase subfamily
01

Overview

Mitochondrial trans-2-enoyl-CoA reductase (MECR) is a mitochondrial enzyme that catalyzes the final step in the mitochondrial fatty acid synthesis (mtFAS) pathway: the NADPH-dependent reduction of trans-2-enoyl thioesters. This reaction is crucial for producing octanoic acid—a precursor for lipoic acid synthesis—which is essential for aerobic metabolism. Mutations or disruptions in MECR are linked to MEPAN syndrome and related neurological disorders. Overexpression can increase PPARα activity. It remains an underexplored drug target.

Other names
CGI-63DYTOABGETR1FASN2BNRBF1OPA16
02

Mechanism of action

N/A

03

Biological functions

Mitochondrial fatty acid synthesis (mtFAS)Octanoic acid productionLipoic acid synthesisProtein lipoylationRegulation of mitochondrial respiratory activity
04

Disease associations

MEPAN syndromeDystonia, childhood-onset with optic atrophy and basal ganglia abnormalitiesOptic atrophy 16Neurodegenerative disease

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