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Gene Review

CHCHD4  -  coiled-coil-helix-coiled-coil-helix domain...

Homo sapiens

Synonyms: Coiled-coil-helix-coiled-coil-helix domain-containing protein 4, FLJ31709, MIA40, Mitochondrial intermembrane space import and assembly protein 40, TIMM40
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High impact information on CHCHD4

  • Furthermore, we show that the twin -CX9C- motif is specifically required for import and stability of MIA40 in mitochondria [1].
  • Taken together, we conclude that the biogenesis and function of MIA40 in the mitochondrial intermembrane space is dependent on redox processes involving conserved cysteine residues [1].
  • A redox pathway (Mia40p and Erv1p) mediates the import of intermembrane space proteins such as the small Tim proteins, Cox17p, and Cox19p, which have disulfide bonds [2].

Associations of CHCHD4 with chemical compounds

  • Partial mutation of this motif affects stable accumulation of MIA40 in the intermembrane space, whereas mutation of all cysteine residues in this motif inhibits import in mitochondria [1].


  1. Functional and mutational characterization of human MIA40 acting during import into the mitochondrial intermembrane space. Hofmann, S., Rothbauer, U., Mühlenbein, N., Baiker, K., Hell, K., Bauer, M.F. J. Mol. Biol. (2005) [Pubmed]
  2. Redox pathways of the mitochondrion. Koehler, C.M., Beverly, K.N., Leverich, E.P. Antioxid. Redox Signal. (2006) [Pubmed]
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