[HTML][HTML] Motor neuron susceptibility in ALS/FTD

AMG Ragagnin, S Shadfar, M Vidal… - Frontiers in …, 2019 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
death of both upper and lower motor neurons (MNs) in the brain, brainstem and spinal cord …

[HTML][HTML] The multi-dimensional roles of astrocytes in ALS

K Yamanaka, O Komine - Neuroscience research, 2018 - Elsevier
Despite significant progress in understanding the molecular and genetic aspects of
amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease characterized by the …

[HTML][HTML] Modulating inflammatory monocytes with a unique microRNA gene signature ameliorates murine ALS

O Butovsky, S Siddiqui, G Gabriely… - The Journal of …, 2012 - Am Soc Clin Investig
Amyotrophic lateral sclerosis (ALS) is a progressive disease associated with neuronal cell
death that is thought to involve aberrant immune responses. Here we investigated the role of …

TDP-43 activates microglia through NF-κB and NLRP3 inflammasome

W Zhao, DR Beers, S Bell, J Wang, S Wen… - Experimental …, 2015 - Elsevier
Transactive response DNA-binding protein-43 (TDP-43) is a multifunctional nucleic acid
binding protein present in ubiquitinated inclusions in tissues of patients with amyotrophic …

Immunity in amyotrophic lateral sclerosis: Blurred lines between excessive inflammation and inefficient immune responses

LC Béland, A Markovinovic, H Jakovac… - Brain …, 2020 - academic.oup.com
Despite wide genetic, environmental and clinical heterogeneity in amyotrophic lateral
sclerosis, a rapidly fatal neurodegenerative disease targeting motoneurons …

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 …

Friends or foes: matrix metalloproteinases and their multifaceted roles in neurodegenerative diseases

M Brkic, S Balusu, C Libert… - Mediators of …, 2015 - Wiley Online Library
Neurodegeneration is a chronic progressive loss of neuronal cells leading to deterioration of
central nervous system (CNS) functionality. It has been shown that neuroinflammation …

[HTML][HTML] From animal models to human disease: a genetic approach for personalized medicine in ALS

V Picher-Martel, PN Valdmanis, PV Gould… - Acta neuropathologica …, 2016 - Springer
Abstract Amyotrophic Lateral Sclerosis (ALS) is the most frequent motor neuron disease in
adults. Classical ALS is characterized by the death of upper and lower motor neurons …

[HTML][HTML] Pharmacological modulation of functional phenotypes of microglia in neurodegenerative diseases

GJ Song, K Suk - Frontiers in aging neuroscience, 2017 - frontiersin.org
Microglia are the resident innate immune cells of the central nervous system that mediate
brain homeostasis maintenance. Microglia-mediated neuroinflammation is a hallmark …

NF‐κB activation in astrocytes drives a stage‐specific beneficial neuroimmunological response in ALS

N Ouali Alami, C Schurr, F Olde Heuvel, L Tang… - The EMBO …, 2018 - embopress.org
Astrocytes are involved in non‐cell‐autonomous pathogenic cascades in amyotrophic
lateral sclerosis (ALS); however, their role is still debated. We show that astrocytic NF‐κB …