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MetabolomicsMetabolomics analysis of plasma from CHCHD10S59L/+ KI mice
Not available
ORGANISM(S): Mus musculus 
ST002435 | MetabolomicsWorkbench
MetabolomicsMetabolomics analysis of heart from CHCHD10S59L/+ KI mice
Not available
ORGANISM(S): Mus musculus 
ST002434 | MetabolomicsWorkbench
MetabolomicsMutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy
Not available
ORGANISM(S): Mus musculus 
ST002068 | MetabolomicsWorkbench
ProteomicsMutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy
Mitochondrial cardiomyopathies are fatal diseases, with no effective treatment. Alterations of heart mitochondrial function activate the mitochondrial integrated stress response (ISRmt), a transcriptional program affecting cell metabolism, mitochondrial biogenesis, and proteostasis. In humans, mutat...
ORGANISM(S): Mus musculus (Mouse) 
2022-03-01 | PXD031444 | Pride
Chchd10 Mitochondria Cardiomyopathy 
ProteomicsHeart mitochondrial proteome of Chchd10(S55L), Oma1(E324A), Chchd10(S55L)/Oma1(E324A) and WT
Mutations in CHCHD10, a mitochondrial protein implicated in proteostasis and cristae maintenance, cause autosomal dominant mitochondrial diseases characterized by cardiomyopathy and neurodegeneration. Heterozygous Chchd10 S55L knock-in mice (modeling the human S59L variant) develop progressive mitoc...
ORGANISM(S): Mus musculus (Mouse) 
2025-11-07 | PXD064045 | Pride
Mouse Solubilisation Mitochondria 
ProteomicsTandem Mass Tag proteomics in CHCHD10 patient cells
Mutations in CHCHD10, coding for a mitochondrial intermembrane space protein, are a rare cause of autosomal dominant amyotrophic lateral sclerosis (ALS). Mutation-specific toxic gain of function or haploinsuffuciency models have been proposed to explain pathogenicity. To decipher the metabolic dysfu...
ORGANISM(S): Homo sapiens (Human) 
2022-04-04 | PXD018806 | Pride
Human Fibroblasts Galactose Mitochondria 
GenomicsCHCHD10 gene KO effect in iPSC drived motor neurons
CHCHD10 gene KO effect in iPSC drived motor neurons
PRJNA552477 | ENA
GenomicsDose-dependent CHCHD10 dysregulation affects motor neuron disease severity and creatine metabolism
Dose-dependent CHCHD10 dysregulation affects motor neuron disease severity and creatine metabolism
PRJNA1157393 | ENA
GenomicsDELE1 mitochondrial integrated stress response to CHCHD10 G58R protein misfolding in striated muscle [RNA-seq]
DELE1 mitochondrial integrated stress response to CHCHD10 G58R protein misfolding in striated muscle [RNA-seq]
PRJNA1141402 | ENA
GenomicsHigh fat diet ameliorates mitochondrial cardiomyopathy in CHCHD10 mutant mice
High fat diet ameliorates mitochondrial cardiomyopathy in CHCHD10 mutant mice
PRJNA1089106 | ENA
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