Results 51 to 60 of about 426,171 (247)

Mitochondrial quality control protects photoreceptors against oxidative stress in the H2O2-induced models of retinal degeneration diseases

open access: yesCell Death and Disease, 2021
Retinal degeneration diseases (RDDs) are common and devastating eye diseases characterized by the degeneration of photoreceptors, which are highly associated with oxidative stress.
Biting Zhou   +7 more
doaj   +1 more source

The eIF2α kinase HRI in innate immunity, proteostasis, and mitochondrial stress [PDF]

open access: yesThe FEBS Journal, 2020
The integrated stress response (ISR) is an evolutionary conserved stress response pathway that leads to a global arrest in translation as well as to the expression of specific genes, such as the transcription factor ATF4, to promote cellular recovery.
Arnoult, Damien   +3 more
openaire   +3 more sources

Mitochondrial Quality Control via Mitochondrial Unfolded Protein Response (mtUPR) in Ageing and Neurodegenerative Diseases

open access: yesBiomolecules, 2023
Mitochondria play a key role in cellular functions, including energy production and oxidative stress regulation. For this reason, maintaining mitochondrial homeostasis and proteostasis (homeostasis of the proteome) is essential for cellular health ...
Paula Cilleros-Holgado   +11 more
doaj   +1 more source

HSF1 is required for induction of mitochondrial chaperones during the mitochondrial unfolded protein response

open access: yesFEBS Open Bio, 2020
The mitochondrial unfolded protein response (UPRmt) is characterized by the transcriptional induction of mitochondrial chaperone and protease genes in response to impaired mitochondrial proteostasis and is regulated by ATF5 and CHOP in mammalian cells ...
Arpit Katiyar   +7 more
doaj   +1 more source

Mitochondria organize the cellular proteostatic response and promote cellular senescence

open access: yesCell Stress, 2019
Mitochondria have relatively independent protein quality control systems, including their own chaperones for protein folding and AAA proteases for protein degradation.
Yanjun Li   +3 more
doaj   +1 more source

HRAS germline mutations impair LKB1/AMPK signaling and mitochondrial homeostasis in Costello syndrome models

open access: yesThe Journal of Clinical Investigation, 2022
Germline mutations that activate genes in the canonical RAS/MAPK signaling pathway are responsible for rare human developmental disorders known as RASopathies.
Laetitia Dard   +22 more
doaj   +1 more source

Mitochondrial autophagy in the sleeping brain

open access: yesFrontiers in Cell and Developmental Biology, 2022
A significant percentage of the mitochondrial mass is replaced on a daily basis via mechanisms of mitochondrial quality control. Through mitophagy (a selective type of autophagy that promotes mitochondrial proteostasis) cells keep a healthy pool of ...
Sofia Mauri   +4 more
doaj   +1 more source

Engineered AAA+ proteases reveal principles of proteolysis at the mitochondrial inner membrane

open access: yesNature Communications, 2016
Human YME1L is a membrane-anchored AAA+ protease that maintains proteostasis in the mitochondrial inner membrane and intermembrane space. Here the authors probe the substrate-binding and degradation activities of YME1L and suggest the existence of ...
Hui Shi   +2 more
doaj   +1 more source

Mitochondrial DNA variants modulate N-formylmethionine, proteostasis and risk of late-onset human diseases. [PDF]

open access: yes, 2021
Mitochondrial DNA (mtDNA) variants influence the risk of late-onset human diseases, but the reasons for this are poorly understood. Undertaking a hypothesis-free analysis of 5,689 blood-derived biomarkers with mtDNA variants in 16,220 healthy donors ...

core   +3 more sources

Altered transcriptome-proteome coupling indicates aberrant proteostasis in Parkinson’s disease

open access: yesiScience, 2023
Summary: Aberrant proteostasis is thought to be implicated in Parkinson’s disease (PD), but patient-derived evidence is scant. We hypothesized that impaired proteostasis is reflected as altered transcriptome-proteome correlation in the PD brain.
Fiona Dick   +4 more
doaj   +1 more source

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