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FLASH GENE
Symbol MCCC1 contributors: mct - updated : 09-03-2007
HGNC name methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
HGNC id 6936
PROTEIN
PHYSICAL PROPERTIES globular
STRUCTURE
motifs/domains
  • N terminal mitochondrial signal peptide (46AA)
  • CPS, N terminal domain
  • ATP binding domain
  • biotine carboxylase domain
  • biotin attachment site
    • mono polymer heteromer , dimer
    HOMOLOGY
    interspecies homolog to murine Mccc1
    Homologene
    FAMILY
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    basic FUNCTION
  • catalyzing one step of degradation of leucine in acetyl COA and aceto-acetate
  • catalyzing carboxylation of 3 methylcrotonyl COA to form 3 methylglutaconyl-COA
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism aminoacid , organic acid
    signaling
    leucine catabolism
    a component
  • heterodimerizing with the beta catalytic subunit
  • heteromultimeric complex that is composed of alpha and beta subunits which are encoded by distinct genes (Chu 2007)
    • INTERACTION
    DNA
    RNA
    small molecule cofactor,
    biotin
    protein
    cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) MCCC1
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS