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CHCHD4, a component of human mitochondria, belongs to a protein family whose members share 6 highly conserved cysteine residues constituting a -CXC-CX(9)C-CX(9)C- motif in the C terminus (Hofmann et al., 2005 [PubMed 16185709]).[supplied by OMIM, Mar 2008]..
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These results suggest that CHCHD4 haploinsufficiency afforded persistent neuroprotection related to reduced release of mitochondrial intermembrane space proteins.
Mechanistically, exercise-induced nuclear transcription factor FOXO3 (show FOXO3 Proteins) binds to the CHCHD4 promoter and represses its expression, preventing the translocation of p53 (show TP53 Proteins) to the mitochondria and thereby increasing p53 (show TP53 Proteins) nuclear localization.
First group to discover the expression of the human CHCHD4 isoforms and first to call the human gene CHCHD4, and to clone and show the expression of its alternatively expressed CHCHD4 isoforms (CHCHD4.1 and CHCHD4.2).
Compared to wild type control littermates, mice with a knockout of CHCHD4 exhibit reduced weight gain when fed a high-fat diet.
Data show that the redox state of cytochrome c oxidase assembly protein 17 (Cox17 (show COX17 Proteins)), mitochondrial membrane transport protein Mia40 and superoxide dismutase 1 (SOD1 (show SOD1 Proteins)) in the cytoplasm were directly observed with in-cell NMR spectroscopy.
our findings suggest that MIA40 reduction contributes to the effects of AIF (show AIFM1 Proteins) deficiency on OXPHOS, as it may impact on the correct assembly and maintenance of the respiratory subunits.
Data indicate that poptosis-inducing factor (AIF (show AIFM1 Proteins)) controls the mitochondrial import of mitochondrial membrane transport protein CHCHD4.
In aggregate these data suggest that the Mia40/lfALR system has a broad sequence specificity and that potential substrates may be protected from adventitious oxidation by kinetic sequestration within the mitochondrial IMS.
Import and oxidative folding of proteins are kinetically and functionally coupled and depend on the expression of Mia40, ALR (show GFER Proteins), and the intracellular glutathione pool.
Data indicate that decreased CHCHD4 expression prevents the mitochondrial translocation of p53 (show TP53 Proteins) while augmenting its nuclear localization and activity.
illustrate a very atypical behaviour for the Mia40 precursor compared to other substrates of the MIA (show MIA Proteins) pathway. By contrast, interaction with Erv1 (show GFER Proteins) occurs after 5 min of import and relies on a more stringent specificity
mitochondrial localization of MIA40 requires sulfhydryl oxidase ALR (show GFER Proteins) in heterologous expression yeast system.
CHCHD4, a component of human mitochondria, belongs to a protein family whose members share 6 highly conserved cysteine residues constituting a -CXC-CX(9)C-CX(9)C- motif in the C terminus (Hofmann et al., 2005
coiled-coil-helix-coiled-coil-helix domain-containing protein 4
, mitochondrial intermembrane space import and assembly protein 40
, mitochondrial intermembrane space import and assembly 40 homolog
, translocase of inner mitochondrial membrane 40 homolog
, mitochondrial intermembrane space import and assembly protein 40-A
, coiled-coil-helix-coiled-coil-helix domain-containing protein 4-A
, coiled-coil-helix-coiled-coil-helix domain containing 4
, mitochondrial intermembrane space import and assembly protein 40-B
, coiled-coil-helix-coiled-coil-helix domain-containing protein 4-B
, Mitochondrial import inner membrane translocase TIM40