Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis
A Prasad, V Bharathi, V Sivalingam… - Frontiers in molecular …, 2019 - frontiersin.org
TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …
Molecular and cellular mechanisms affected in ALS
L Le Gall, E Anakor, O Connolly… - Journal of personalized …, 2020 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a terminal late-onset condition characterized by the
loss of upper and lower motor neurons. Mutations in more than 30 genes are associated to …
loss of upper and lower motor neurons. Mutations in more than 30 genes are associated to …
Mitochondrial cristae architecture protects against mtDNA release and inflammation
Mitochondrial damage causes mitochondrial DNA (mtDNA) release to activate the type I
interferon (IFN-I) response via the cGAS-STING pathway. mtDNA-induced inflammation …
interferon (IFN-I) response via the cGAS-STING pathway. mtDNA-induced inflammation …
The debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: a resolution in sight?
RC Hergesheimer, AA Chami, DR De Assis, P Vourc'h… - Brain, 2019 - academic.oup.com
Transactive response DNA-binding protein-43 (TDP-43) is an RNA/DNA binding protein that
forms phosphorylated and ubiquitinated aggregates in the cytoplasm of motor neurons in …
forms phosphorylated and ubiquitinated aggregates in the cytoplasm of motor neurons in …
Nutritional and metabolic factors in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disease that is
classically thought to impact the motor system. Over the past 20 years, research has started …
classically thought to impact the motor system. Over the past 20 years, research has started …
CHCHD2 and CHCHD10 regulate mitochondrial dynamics and integrated stress response
Y Ruan, J Hu, Y Che, Y Liu, Z Luo, J Cheng… - Cell Death & …, 2022 - nature.com
Mitochondrial dysfunction is becoming one of the main pathology factors involved in the
etiology of neurological disorders. Recently, mutations of the coiled-coil-helix-coiled-coil …
etiology of neurological disorders. Recently, mutations of the coiled-coil-helix-coiled-coil …
3D reconstruction of murine mitochondria reveals changes in structure during aging linked to the MICOS complex
During aging, muscle gradually undergoes sarcopenia, the loss of function associated with
loss of mass, strength, endurance, and oxidative capacity. However, the 3D structural …
loss of mass, strength, endurance, and oxidative capacity. However, the 3D structural …
MICOS and the mitochondrial inner membrane morphology–when things get out of shape
I Mukherjee, M Ghosh, M Meinecke - FEBS letters, 2021 - Wiley Online Library
Mitochondria play a key role in cellular signalling, metabolism and energetics. Proper
architecture and remodelling of the inner mitochondrial membrane are essential for efficient …
architecture and remodelling of the inner mitochondrial membrane are essential for efficient …
Modelling amyotrophic lateral sclerosis in rodents
TW Todd, L Petrucelli - Nature Reviews Neuroscience, 2022 - nature.com
The efficient study of human disease requires the proper tools, one of the most crucial of
which is an accurate animal model that faithfully recapitulates the human condition. The …
which is an accurate animal model that faithfully recapitulates the human condition. The …
The 'mitochondrial contact site and cristae organising system'(MICOS) in health and human disease
MJ Eramo, V Lisnyak, LE Formosa… - The Journal of …, 2020 - academic.oup.com
The 'mitochondrial contact site and cristae organising system'(MICOS) is an essential protein
complex that promotes the formation, maintenance and stability of mitochondrial cristae. As …
complex that promotes the formation, maintenance and stability of mitochondrial cristae. As …