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 …

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 …

Mitochondrial cristae architecture protects against mtDNA release and inflammation

B He, H Yu, S Liu, H Wan, S Fu, S Liu, J Yang, Z Zhang… - Cell Reports, 2022 - cell.com
Mitochondrial damage causes mitochondrial DNA (mtDNA) release to activate the type I
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 …

Nutritional and metabolic factors in amyotrophic lateral sclerosis

A Ludolph, L Dupuis, E Kasarskis, F Steyn… - Nature Reviews …, 2023 - nature.com
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 …

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 …

3D reconstruction of murine mitochondria reveals changes in structure during aging linked to the MICOS complex

Z Vue, E Garza‐Lopez, K Neikirk, P Katti, L Vang… - Aging cell, 2023 - Wiley Online Library
During aging, muscle gradually undergoes sarcopenia, the loss of function associated with
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 …

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 …

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 …