Disturbed mitochondrial dynamics and neurodegenerative disorders
Mitochondria form a highly interconnected tubular network throughout the cell via a dynamic
process, with mitochondrial segments fusing and breaking apart continuously. Strong …
process, with mitochondrial segments fusing and breaking apart continuously. Strong …
New roles for mitochondrial proteases in health, ageing and disease
Recent advances in mitochondrial biology have revealed the high diversity and complexity
of proteolytic enzymes that regulate mitochondrial function. We have classified mitochondrial …
of proteolytic enzymes that regulate mitochondrial function. We have classified mitochondrial …
Targeting mitochondrial structure sensitizes acute myeloid leukemia to venetoclax treatment
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 …
selective BCL2 inhibitor, has received FDA approval for the treatment of AML. However …
Mitochondrial proteases: multifaceted regulators of mitochondrial plasticity
Mitochondria are essential metabolic hubs that dynamically adapt to physiological demands.
More than 40 proteases residing in different compartments of mitochondria, termed …
More than 40 proteases residing in different compartments of mitochondria, termed …
Calcium and regulation of the mitochondrial permeability transition
Recent years have seen renewed interest in the permeability transition pore, a high
conductance channel responsible for permeabilization of the inner mitochondrial …
conductance channel responsible for permeabilization of the inner mitochondrial …
The m-AAA protease associated with neurodegeneration limits MCU activity in mitochondria
Mutations in subunits of mitochondrial m-AAA proteases in the inner membrane cause
neurodegeneration in spinocerebellar ataxia (SCA28) and hereditary spastic paraplegia …
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
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 …
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 …
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 …
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 …
are required for mitochondrial morphogenesis, neuronal survival, and normal lifespan. Here …