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FLASH GENE
Symbol PMPCB contributors: mct - updated : 16-05-2018
HGNC name peptidase (mitochondrial processing) beta
HGNC id 9119
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
zinc binding region
conjugated PhosphoP
mono polymer heteromer , dimer
HOMOLOGY
Homologene
FAMILY
  • insulinase family
  • peptidase M16 family
  • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,mitochondria,matrix
    intracellular,cytoplasm,organelle,membrane
    basic FUNCTION
  • acting as a mitochondrial processing peptidase
  • catalytic subunit cleaving presequences from mitochondrial protein precursors
  • catalytic subunit of the essential mitochondrial processing protease (MPP), which is required for maturation of the majority of mitochondrial precursor proteins
  • CELLULAR PROCESS protein, post translation
    PHYSIOLOGICAL PROCESS
    text involved in transit peptide
    PATHWAY
    metabolism
    signaling
    a component heterodimer of alpha and beta subunits
    INTERACTION
    DNA
    RNA
    small molecule metal binding,
  • Zn2+ (per subunit)
  • protein
  • mitochondrial processing peptidase PMPCB generates an intermediate form of AFG3L2 that is matured autocatalytically
  • new role for PMPCB in PINK1 import and mitochondrial quality control via the PINK1–Parkin pathway
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) MMDS6
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS