Recombinant Human Methylmalonyl-CoA mutase, mitochondrial (MMUT)

Uniprot Id

P22033

Target Species

Human

Target Name

MMUT

Target Full Name

Methylmalonyl-CoA mutase, mitochondrial

Target Function

Catalyzes the reversible isomerization of methylmalonyl-CoA (MMCoA) (generated from branched-chain amino acid metabolism and degradation of dietary odd chain fatty acids and cholesterol) to succinyl-CoA (3-carboxypropionyl-CoA), a key intermediate of the tricarboxylic acid cycle.

Target Involvement

Methylmalonic aciduria type mut (MMAM)

Target Subcellular Location

Mitochondrion matrix. Mitochondrion. Cytoplasm.

Target Protein Families

Methylmalonyl-CoA mutase family

Target Research Area

Signal Transduction

Target Synonyms

MCM; Methylmalonyl CoA isomerase ; Methylmalonyl CoA mutase mitochondrial; Methylmalonyl Coenzyme A mutase; Methylmalonyl-CoA isomerase; Methylmalonyl-CoA mutase; mitochondrial; Mut; MUTA_HUMAN

Target Background

This gene encodes the mitochondrial enzyme methylmalonyl Coenzyme A mutase. In humans, the product of this gene is a vitamin B12-dependent enzyme which catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA, while in other species this enzyme may have different functions. Mutations in this gene may lead to various types of methylmalonic aciduria.