Decoding ALS: from genes to mechanism

JP Taylor, RH Brown Jr, DW Cleveland - Nature, 2016 - nature.com
Amyotrophic lateral sclerosis (ALS) is a progressive and uniformly fatal neurodegenerative
disease. A plethora of genetic factors have been identified that drive the degeneration of …

Development of multifunctional molecules as potential therapeutic candidates for Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis in the …

MG Savelieff, G Nam, J Kang, HJ Lee, M Lee… - Chemical …, 2018 - ACS Publications
Neurodegenerative diseases pose a substantial socioeconomic burden on society.
Unfortunately, the aging world population and lack of effective cures foreshadow a negative …

Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis

SC Ling, M Polymenidou, DW Cleveland - Neuron, 2013 - cell.com
Breakthrough discoveries identifying common genetic causes for amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …

Spreading of pathology in neurodegenerative diseases: a focus on human studies

J Brettschneider, KD Tredici, VMY Lee… - Nature Reviews …, 2015 - nature.com
The progression of many neurodegenerative diseases is thought to be driven by the
template-directed misfolding, seeded aggregation and cell–cell transmission of …

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 …

[HTML][HTML] VDAC1 at the intersection of cell metabolism, apoptosis, and diseases

V Shoshan-Barmatz, A Shteinfer-Kuzmine, A Verma - Biomolecules, 2020 - mdpi.com
The voltage-dependent anion channel 1 (VDAC1) protein, is an important regulator of
mitochondrial function, and serves as a mitochondrial gatekeeper, with responsibility for …

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

HX Deng, W Chen, ST Hong, KM Boycott, GH Gorrie… - Nature, 2011 - nature.com
Amyotrophic lateral sclerosis (ALS) is a paralytic and usually fatal disorder caused by motor-
neuron degeneration in the brain and spinal cord. Most cases of ALS are sporadic but about …

Superoxide dismutase 1 in health and disease: how a frontline antioxidant becomes neurotoxic

BG Trist, JB Hilton, DJ Hare, PJ Crouch… - Angewandte Chemie …, 2021 - Wiley Online Library
Cu/Zn superoxide dismutase (SOD1) is a frontline antioxidant enzyme catalysing superoxide
breakdown and is important for most forms of eukaryotic life. The evolution of aerobic …

Pathomechanisms of TDP‐43 in neurodegeneration

J Gao, L Wang, ML Huntley, G Perry… - Journal of …, 2018 - Wiley Online Library
Neurodegeneration, a term that refers to the progressive loss of structure and function of
neurons, is a feature of many neurodegenerative diseases such as amyotrophic lateral …

Rodent models of amyotrophic lateral sclerosis

T Philips, JD Rothstein - Current protocols in pharmacology, 2015 - Wiley Online Library
Abstract Amyotrophic Lateral Sclerosis (ALS) is a motor neuron disease affecting upper and
lower motor neurons in the central nervous system. Patients with ALS develop extensive …