[HTML][HTML] The role of mitochondria in amyotrophic lateral sclerosis

EF Smith, PJ Shaw, KJ De Vos - Neuroscience letters, 2019 - Elsevier
Mitochondria are unique organelles that are essential for a variety of cellular processes
including energy metabolism, calcium homeostasis, lipid biosynthesis, and apoptosis …

[HTML][HTML] Autophagy dysregulation in ALS: when protein aggregates get out of hand

N Ramesh, UB Pandey - Frontiers in molecular neuroscience, 2017 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that results from the
loss of upper and lower motor neurons. One of the key pathological hallmarks in diseased …

[HTML][HTML] TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response

P Wang, J Deng, J Dong, J Liu, EH Bigio… - PLoS …, 2019 - journals.plos.org
Mutations in or dys-regulation of the TDP-43 gene have been associated with TDP-43
proteinopathy, a spectrum of neurodegenerative diseases including Frontotemporal Lobar …

Functional significance of TDP-43 mutations in disease

E Buratti - Advances in genetics, 2015 - Elsevier
At present, there are very few therapeutic options for patients affected by amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD). However, almost all patients affected by …

Oxidative stress and mitochondrial damage in the pathogenesis of ALS: New perspectives

F Bozzo, A Mirra, MT Carrì - Neuroscience letters, 2017 - Elsevier
This review attempts to reconcile the present dual view of the mechanisms operating in
Amyotrophic Lateral Sclerosis (ALS). On one side, oxidative stress, mitochondrial damage …

[HTML][HTML] Nuclear poly (ADP-ribose) activity is a therapeutic target in amyotrophic lateral sclerosis

L McGurk, J Mojsilovic-Petrovic, VM Van Deerlin… - Acta neuropathologica …, 2018 - Springer
Amyotrophic lateral sclerosis (ALS) is a devastating and fatal motor neuron disease.
Diagnosis typically occurs in the fifth decade of life and the disease progresses rapidly …

[HTML][HTML] Dysregulation of RNA splicing in tauopathies

DJ Apicco, C Zhang, B Maziuk, L Jiang, HI Ballance… - Cell reports, 2019 - cell.com
Pathological aggregation of RNA binding proteins (RBPs) is associated with dysregulation
of RNA splicing in PS19 P301S tau transgenic mice and in Alzheimer's disease brain …

[HTML][HTML] Nicotinamide as a foundation for treating neurodegenerative disease and metabolic disorders

K Maiese - Current neurovascular research, 2021 - ncbi.nlm.nih.gov
Neurodegenerative disorders impact more than one billion individuals worldwide and are
intimately tied to metabolic disease that can affect another nine hundred individuals …

Pathways to mitochondrial dysfunction in ALS pathogenesis

MT Carrì, N D'Ambrosi, M Cozzolino - Biochemical and biophysical …, 2017 - Elsevier
Alterations in the structure and functions of mitochondria are a typical trait of Amyotrophic
Lateral Sclerosis, a neurodegenerative disease characterized by a prominent degeneration …

[HTML][HTML] A loss-of-function mutation in human Oxidation Resistance 1 disrupts the spatial–temporal regulation of histone arginine methylation in neurodevelopment

X Lin, W Wang, M Yang, N Damseh, MML de Sousa… - Genome Biology, 2023 - Springer
Abstract Background Oxidation Resistance 1 (OXR1) gene is a highly conserved gene of the
TLDc domain-containing family. OXR1 is involved in fundamental biological and cellular …