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FLASH GENE
Symbol CHCHD3 contributors: mct - updated : 21-11-2017
HGNC name coiled-coil-helix-coiled-coil-helix domain containing 3
HGNC id 21906
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • N-terminal myristoylation motif, that promotes binding of CHCHD3 to the outer membrane
  • a DUF737 domain (domain of unknown function)
  • a coiled-coil helix-coiled-coil helix (CHCH) domain , a CHCH domain with twin CX(9)C motifs, that translocates the protein across the outer membrane
  • HOMOLOGY
    interspecies homolog to murine Chchd3
    Homologene
    FAMILY
    CATEGORY structural protein
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,membrane
    intracellular,nucleus
    text
  • peripheral protein of the inner mitochondrial membrane
  • basic FUNCTION
  • peripheral protein of the inner membrane facing the intermembrane space
  • scaffolding protein that stabilizes protein complexes involved in maintaining crista architecture and protein import and is thus essential for maintaining mitochondrial structure and function
  • essential role in maintaining crista junctions architecture, crista morphology, and mitochondrial function
  • could be one of the key proteins that coordinate protein import and mitochondrial morphology, the two essential processes involved in proper functioning of mitochondria
  • integrating role of MINOS subunits (IMMT, MINOS1, and CHCHD3) in the structural organization of the organelle in mitochondria
  • CHCHD3 oxidation plays a role in MICOS assembly and the maintenance of the proper inner membrane morphology, but is not essential for its integration into the MICOS complex
  • is likely a redox-dependent regulator of MICOS function
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • most important subunits of the MIB complex in human mitochondria are IMMT, CHCHD3 and an outer membrane component SAMM50
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • PRKACA substrate
  • interacts with mitofilin and OPA1, which regulate crista morphology, and the outer membrane protein SAMM50, which regulates import and assembly of beta-barrel proteins on the outer membrane
  • CHCHD3 binds to CHCHD4 for further folding and assembly into macromolecular complexes
  • direct interaction between IMMT and CHCHD3, which is crucial for their stabilization in mammals
  • SAMM50 incompletely overlap with CHCHD3- or IMMT-positive domains, most notably at the crista junctions (CJs)
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    ANIMAL & CELL MODELS