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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
DNA
TYPE like-sequence
STRUCTURE 297.30 kb     8 Exon(s)
MAPPING cloned Y linked N status provisional
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
8 - 1622 - 227 - 2011 21081504
9 - 1722 - 232 - 2011 21081504
5 - 1187 - 138 - 2011 21081504
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Nervousbrain   highly Homo sapiens
Reproductivefemale systembreastmammary gland  
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Muscularstriatumskeletal highly
cells
SystemCellPubmedSpeciesStageRna symbol
Nervousneuron Homo sapiens
cell lineage
cell lines
fluid/secretion
at STAGE
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