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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].. Additionally we are shipping CHCHD4 Antibodies (56) and and many more products for this protein.
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To test whether differences in the ratio between CHCHD4 and ALR might explain tissue-specific differences in the CHCHD4 redox state, we determined the molar ratio of both proteins in different mouse tissues. Surprisingly, ALR is superstoichiometric over CHCHD4 in most tissues.
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 binds to the CHCHD4 promoter and represses its expression, preventing the translocation of p53 to the mitochondria and thereby increasing p53 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), mitochondrial membrane transport protein Mia40 and superoxide dismutase 1 (SOD1) in the cytoplasm were directly observed with in-cell NMR spectroscopy.
our findings suggest that MIA40 reduction contributes to the effects of AIF deficiency on OXPHOS, as it may impact on the correct assembly and maintenance of the respiratory subunits.
results demonstrate an indispensable role for hMIA40 for the export of Fe-S clusters from mitochondria.
Data indicate that poptosis-inducing factor (AIF) 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, and the intracellular glutathione pool.
Data indicate that decreased CHCHD4 expression prevents the mitochondrial translocation of p53 while augmenting its nuclear localization and activity.
illustrate a very atypical behaviour for the Mia40 precursor compared to other substrates of the MIA pathway. By contrast, interaction with Erv1 occurs after 5 min of import and relies on a more stringent specificity
mitochondrial localization of MIA40 requires sulfhydryl oxidase ALR in heterologous expression yeast system.
Molecular recognition and substrate mimicry drive the electron-transfer process between MIA40 and ALR.
biogenesis and function of MIA40 in the mitochondrial intermembrane space is dependent on redox processes involving conserved cysteine residues
After passage across the translocase of the mitochondrial outer membrane Erv1 interacts via disulfide bonds with Mia40.
analysis of how mitochondrial biogenesis switches the sorting pathway of the intermembrane space receptor Mia40
Catalysis involves a flow of reducing equivalents from the reduced CxC cysteine motif of Mia40 to distal and then proximal CxxC motifs of long-form ALR to the flavin ring and, finally, to cytochrome c or molecular oxygen.
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