Mechanisms of mitochondrial respiratory adaptation

CF Bennett, P Latorre-Muro, P Puigserver - Nature Reviews Molecular …, 2022 - nature.com
Mitochondrial energetic adaptations encompass a plethora of conserved processes that
maintain cell and organismal fitness and survival in the changing environment by adjusting …

[HTML][HTML] The overlapping genetics of amyotrophic lateral sclerosis and frontotemporal dementia

YA Abramzon, P Fratta, BJ Traynor… - Frontiers in neuroscience, 2020 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two diseases
that form a broad neurodegenerative continuum. Considerable effort has been made to …

Resveratrol and brain mitochondria: a review

FR Jardim, FT de Rossi, MX Nascimento… - Molecular …, 2018 - Springer
Abstract Resveratrol (3, 4', 5-trihydroxystilbene; C 14 H 12 O 3) is a polyphenolic phytoalexin
found in grapes, berries, peanuts, and wines. Resveratrol has been viewed as an …

[HTML][HTML] Reversal of phenotypic abnormalities by CRISPR/Cas9-mediated gene correction in Huntington disease patient-derived induced pluripotent stem cells

X Xu, Y Tay, B Sim, SI Yoon, Y Huang, J Ooi, KH Utami… - Stem cell reports, 2017 - cell.com
Huntington disease (HD) is a dominant neurodegenerative disorder caused by a CAG
repeat expansion in HTT. Here we report correction of HD human induced pluripotent stem …

[HTML][HTML] A cold-stress-inducible PERK/OGT axis controls TOM70-assisted mitochondrial protein import and cristae formation

P Latorre-Muro, KE O'Malley, CF Bennett, EA Perry… - Cell metabolism, 2021 - cell.com
The architecture of cristae provides a spatial mitochondrial organization that contains
functional respiratory complexes. Several protein components including OPA1 and MICOS …

[HTML][HTML] Decoding metabolic signatures in Alzheimer's disease: A mitochondrial perspective

D Bano, D Ehninger, G Bagetta - Cell Death Discovery, 2023 - nature.com
Alzheimer's disease (AD) is one of the most prevalent age-related neurodegenerative
diseases and accounts for the majority of dementia cases worldwide. Tremendous ongoing …

Intrinsically disordered proteins and proteins with intrinsically disordered regions in neurodegenerative diseases

O Coskuner-Weber, O Mirzanli, VN Uversky - Biophysical Reviews, 2022 - Springer
Many different intrinsically disordered proteins and proteins with intrinsically disordered
regions are associated with neurodegenerative diseases. These types of proteins including …

[HTML][HTML] AIFM1 beyond cell death: An overview of this OXPHOS-inducing factor in mitochondrial diseases

L Wischhof, E Scifo, D Ehninger, D Bano - EBioMedicine, 2022 - thelancet.com
Apoptosis-inducing factor (AIF) is a mitochondrial intermembrane space flavoprotein with
diverse functions in cellular physiology. In this regard, a large number of studies have …

The mitochondrial intermembrane space: the most constricted mitochondrial sub-compartment with the largest variety of protein import pathways

R Edwards, R Eaglesfield, K Tokatlidis - Open biology, 2021 - royalsocietypublishing.org
The mitochondrial intermembrane space (IMS) is the most constricted sub-mitochondrial
compartment, housing only about 5% of the mitochondrial proteome, and yet is endowed …

Loss of CHCHD10–CHCHD2 complexes required for respiration underlies the pathogenicity of a CHCHD10 mutation in ALS

IR Straub, A Janer, W Weraarpachai… - Human molecular …, 2018 - academic.oup.com
Coiled-helix coiled-helix domain containing protein 10 (CHCHD10) and its paralogue
CHCHD2 belong to a family of twin CX9C motif proteins, most of which localize to the …