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ProteomicsBiallelic variants in CHCHD4 are associated with combined OXPHOS defect leading to mitochondrial disease
Mitochondrial disorders show remarkable clinical and genetic heterogeneity, and result from genetic variants in either mitochondrial-encoded or nuclear-encoded genes. CHCHD4 is a component of the mitochondrial import and assembly (MIA) pathway that imports small cysteine-containing substrates such a...
ORGANISM(S): Homo sapiens (Human) 
2026-01-21 | PXD069027 | Pride
Chchd4 Timstof ht Evosep Mitochondrial disorders 
GenomicsCHCHD4-TRIAP1 regulation of innate immune signaling mediates skeletal muscle adaptation to exercise
CHCHD4-TRIAP1 regulation of innate immune signaling mediates skeletal muscle adaptation to exercise
PRJNA1029906 | ENA
UnknownElevated CHCHD4 orchestrates mitochondrial oxidative phosphorylation to disturb hypoxic pulmonary hypertension.
Not available
S-EPMC10339524 | biostudies-literature
UnknownThe mitochondrial oxidoreductase CHCHD4 is present in a semi-oxidized state in vivo.
Not available
S-EPMC6007816 | biostudies-literature
UnknownUSF1-CHCHD4 axis promotes lung adenocarcinoma progression partially via activating the MYC pathway.
Not available
S-EPMC9732179 | biostudies-literature
UnknownCHCHD4 regulates tumour proliferation and EMT-related phenotypes, through respiratory chain-mediated metabolism.
Not available
S-EPMC6632184 | biostudies-literature
UnknownNADH-bound AIF activates the mitochondrial CHCHD4/MIA40 chaperone by a substrate-mimicry mechanism.
Not available
S-EPMC11832770 | biostudies-literature
TranscriptomicsCHCHD4-TRIAP1 regulation of innate immune signaling mediates skeletal muscle adaptation to exercise
Exercise training can stimulate the formation of fatty acid oxidizing slow-twitch skeletal muscle fibers which are inversely correlated with obesity, but the molecular mechanism underlying this transformation requires further elucidation. Here, we report that the downregulation of the mitochondrial ...
ORGANISM(S): Mus musculus 
2024-01-04 | GSE245760 | GEO
UnknownCHCHD4 links AIF to the biogenesis of respiratory chain complex I.
Not available
S-EPMC4905392 | biostudies-literature
UnknownCHCHD4 confers metabolic vulnerabilities to tumour cells through its control of the mitochondrial respiratory chain.
Not available
S-EPMC6404347 | biostudies-literature
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