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FLASH GENE
Symbol MUT contributors: mct - updated : 23-04-2013
HGNC name methylmalonyl Coenzyme A mutase
HGNC id 7526
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a 32 leader sequence A
  • N terminal CoA binding domain
  • TIM-barrel domain (beta/alpha 8 barrel domain)
  • a C-terminal domain 5 cofactor-binding domain with a large beta sheet consisting of five parallel strands
    • mono polymer homomer , dimer
    isoforms Precursor translocated in the mitochondria and cleaved by a matrix peptidase to form the mature subunit (718 AA)
    HOMOLOGY
    Homologene
    FAMILY methylmalonyl-Coa mutase family
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,matrix
    basic FUNCTION
  • conversion of L-methylmalonyl-CoA to succinyl-CoA in the propionyl-CoA catabolic pathway toward the Krebs cycle
  • involved in the degradation of several amino acids (isoleucine, valine, methionine and threonine) odd-chain fatty acids and cholesterol via propionyl-CoA
    • to the tricarboxylic acid cycle
    CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism aminoacid , organic acid
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule cofactor,
  • adenosylcobalamin (vitamin B12 dependent)
    • protein
  • MMAA exhibits GTPase activity that is modulated by MUT
  • MMAA plays dual roles in MUT activity
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) MUT
    related resource MITOP database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS