Methylmalonyl-CoA mutase, mitochondrial

BE0000777ProteinHumansMMUT

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. It is acted on by 4 drugs in DrugBank.

Gene name
MMUT
UniProtKB entry
P22033Swiss-Prot
Cellular location
Mitochondrion matrix
Residues / weight
750 residues · 83,134 Da
Also known as
5.4.99.2 · MCM · Methylmalonyl-CoA isomerase · MUT

Resolves to

Protein
UniProtP22033UniProt nameMUTA_HUMAN
Gene
HGNCHGNC:7526NCBI Gene4594GenBank187452GeneCardsMMUT
Pharmacology
KEGGhsa:4594
Structure
PDB2XIJPDB2XIQPDB3BIC
Drugs

What you can answer from here — as of September 07, 2019

4DrugsApproved to experimental, with action, bond type and pharmacological action
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7GO termsFunction, process and component, as structured annotations
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2Pfam domainsDomain architecture mapped to Pfam families and clans
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40+ReferencesStructured and connected to the statements they support
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Which approved drugs act on Methylmalonyl-CoA mutase, mitochondrial, how selective are they, and which programs against it are still in active trials?

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