Disturbed mitochondrial dynamics and neurodegenerative disorders

F Burté, V Carelli, PF Chinnery… - Nature reviews …, 2015 - nature.com
Mitochondria form a highly interconnected tubular network throughout the cell via a dynamic
process, with mitochondrial segments fusing and breaking apart continuously. Strong …

New roles for mitochondrial proteases in health, ageing and disease

PM Quirós, T Langer, C López-Otín - Nature reviews Molecular cell …, 2015 - nature.com
Recent advances in mitochondrial biology have revealed the high diversity and complexity
of proteolytic enzymes that regulate mitochondrial function. We have classified mitochondrial …

Targeting mitochondrial structure sensitizes acute myeloid leukemia to venetoclax treatment

X Chen, C Glytsou, H Zhou, S Narang, DE Reyna… - Cancer discovery, 2019 - AACR
The BCL2 family plays important roles in acute myeloid leukemia (AML). Venetoclax, a
selective BCL2 inhibitor, has received FDA approval for the treatment of AML. However …

Mitochondrial proteases: multifaceted regulators of mitochondrial plasticity

S Deshwal, KU Fiedler, T Langer - Annual review of …, 2020 - annualreviews.org
Mitochondria are essential metabolic hubs that dynamically adapt to physiological demands.
More than 40 proteases residing in different compartments of mitochondria, termed …

Calcium and regulation of the mitochondrial permeability transition

V Giorgio, L Guo, C Bassot, V Petronilli, P Bernardi - Cell calcium, 2018 - Elsevier
Recent years have seen renewed interest in the permeability transition pore, a high
conductance channel responsible for permeabilization of the inner mitochondrial …

The m-AAA protease associated with neurodegeneration limits MCU activity in mitochondria

T König, SE Tröder, K Bakka, A Korwitz… - Molecular cell, 2016 - cell.com
Mutations in subunits of mitochondrial m-AAA proteases in the inner membrane cause
neurodegeneration in spinocerebellar ataxia (SCA28) and hereditary spastic paraplegia …

Tissue-specific loss of DARS2 activates stress responses independently of respiratory chain deficiency in the heart

SA Dogan, C Pujol, P Maiti, A Kukat, S Wang… - Cell metabolism, 2014 - cell.com
Adaptive stress responses activated upon mitochondrial dysfunction are assumed to arise in
order to counteract respiratory chain deficiency. Here, we demonstrate that loss of DARS2 …

The classification of autosomal recessive cerebellar ataxias: a consensus statement from the society for research on the cerebellum and ataxias task force

M Beaudin, A Matilla-Dueñas, BW Soong, JL Pedroso… - The Cerebellum, 2019 - Springer
There is currently no accepted classification of autosomal recessive cerebellar ataxias, a
group of disorders characterized by important genetic heterogeneity and complex …

Regulation of mitochondrial proteostasis by the proton gradient

M Patron, D Tarasenko, H Nolte, L Kroczek… - The EMBO …, 2022 - embopress.org
Mitochondria adapt to different energetic demands reshaping their proteome. Mitochondrial
proteases are emerging as key regulators of these adaptive processes. Here, we use a …

DNAJC19, a mitochondrial cochaperone associated with cardiomyopathy, forms a complex with prohibitins to regulate cardiolipin remodeling

R Richter-Dennerlein, A Korwitz, M Haag, T Tatsuta… - Cell metabolism, 2014 - cell.com
Prohibitins form large protein and lipid scaffolds in the inner membrane of mitochondria that
are required for mitochondrial morphogenesis, neuronal survival, and normal lifespan. Here …