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MetabolomicsTargeted degradation of the α-ketoglutarate dehydrogenase mediated by a mitochondrial DNAJC protein regulates metabolism

Mitochondrial heat shock proteins and co-chaperones play essential roles in maintaining proteostasis. They are renowned for regulating unfolded proteins, usually without specific target preferences. In this study, we identified T cell activation inhibitor, mitochondria (TCAIM), as a novel DNAJC-t...

2024-12-03 | MTBLS10440 | MetaboLights
TranscriptomicsMitochondrial unfolded protein response controls matrix pre-RNA processing and translation
The mitochondrial matrix is unique in that it must integrate folding and assembly of proteins derived from nuclear and mitochondrial genomes. In C. elegans, the mitochondrial unfolded protein response (UPRmt) senses matrix protein misfolding and induces a program of nuclear gene expression, includin...
ORGANISM(S): Homo sapiens 
2016-06-28 | E-GEOD-75247 | biostudies-arrayexpress
TranscriptomicsMitochondrial unfolded protein response controls matrix pre-RNA processing and translation
The mitochondrial matrix is unique in that it must integrate folding and assembly of proteins derived from nuclear and mitochondrial genomes. In C. elegans, the mitochondrial unfolded protein response (UPRmt) senses matrix protein misfolding and induces a program of nuclear gene expression, includin...
ORGANISM(S): Homo sapiens 
2016-06-28 | E-GEOD-75410 | biostudies-arrayexpress
GenomicsAnakinra restores cellular proteostasis by coupling mitochondrial redox balance to autophagy
Anakinra restores cellular proteostasis by coupling mitochondrial redox balance to autophagy
PRJNA780423 | ENA
ProteomicsTemporal landscape of mitochondrial proteostasis governed by the UPRmt
When mitochondrial proteostasis breaks down, quality control pathways including the UPRmt and PINK1/Parkin mitophagy are activated in response. However, beyond the upregulation of chaperones and proteases, we have a limited understanding of how the UPRmt remodels and restores damaged mito-proteomes....
ORGANISM(S): Homo sapiens (Human) 
2023-09-27 | PXD038403 | Pride
Mitochondria  metabolism  oxphos  proteostasis  uprmt  mitophagy  pink1  parkin 
ProteomicsHAX1 controls mitochondrial proteostasis
The relevance of mitochondrial proteostasis for the differentiation and function of hematopoietic and immune cells is unknown. Dissecting the HCLS1-associated protein X-1 (HAX1) –related protein network, we define a functional CLPB-HAX1-PRKD2-HSP27 axis with critical importance for the differentiati...
ORGANISM(S): Homo sapiens (Human) 
2022-05-19 | PXD023790 | Pride
Inborn errors of immunity Severe congenital neutropenia Protein quality control Hematopoiesis 
UnknownEnhancing mitochondrial proteostasis reduces amyloid-β proteotoxicity.
Not available
S-EPMC5730497 | biostudies-literature
UnknownCaloric Restriction Induces MicroRNAs to Improve Mitochondrial Proteostasis.
Not available
S-EPMC6614116 | biostudies-literature
ProteomicsMitochondrial Proteostasis Requires Genes Encoded in a Neurodevelopmental Syndrome Locus
Eukaryotic cells maintain proteostasis through mechanisms that require cytoplasmic and mitochondrial translation. Genetic defects affecting cytoplasmic translation perturb synapse development, neurotransmission, and are causative of neurodevelopmental disorders such as Fragile X syndrome. In contras...
ORGANISM(S): Mus musculus (Mouse) 
2022-02-17 | PXD025289 | Pride
Faims Orbitrap Lc-msms 
UnknownMitochondrial Proteostasis Requires Genes Encoded in a Neurodevelopmental Syndrome Locus.
Not available
S-EPMC8336702 | biostudies-literature
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