Neuromuscular junction dysfunction in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by
progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier …
progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier …
Ependymal cells and neurodegenerative disease: outcomes of compromised ependymal barrier function
DG Nelles, LN Hazrati - Brain communications, 2022 - academic.oup.com
Within the central nervous system, ependymal cells form critical components of the blood-
cerebrospinal fluid barrier and the cerebrospinal fluid-brain barrier. These barriers provide …
cerebrospinal fluid barrier and the cerebrospinal fluid-brain barrier. These barriers provide …
Chitotriosidase-a putative biomarker for sporadic amyotrophic lateral sclerosis
AM Varghese, A Sharma, P Mishra, K Vijayalakshmi… - Clinical proteomics, 2013 - Springer
Background Potential biomarkers to aid diagnosis and therapy need to be identified for
Amyotrophic Lateral Sclerosis, a progressive motor neuronal degenerative disorder. The …
Amyotrophic Lateral Sclerosis, a progressive motor neuronal degenerative disorder. The …
Protein homeostasis in amyotrophic lateral sclerosis: therapeutic opportunities?
CP Webster, EF Smith, PJ Shaw… - Frontiers in molecular …, 2017 - frontiersin.org
Protein homeostasis (proteostasis), the correct balance between production and
degradation of proteins, is essential for the health and survival of cells. Proteostasis requires …
degradation of proteins, is essential for the health and survival of cells. Proteostasis requires …
[HTML][HTML] In vitro and in vivo models of amyotrophic lateral sclerosis: an updated overview
AM Gois, DMF Mendonça, MAM Freire… - Brain Research Bulletin, 2020 - Elsevier
Abstract Amyotrophic Lateral Sclerosis (ALS) is a progressive, neurodegenerative disease
characterized by loss of upper motor neurons (UMN) and lower motor neurons (LMN) …
characterized by loss of upper motor neurons (UMN) and lower motor neurons (LMN) …
[HTML][HTML] Intercellular transmission of pathogenic proteins in ALS: Exploring the pathogenic wave
FJ Arnold, AD Nguyen, RS Bedlack, CL Bennett… - Neurobiology of …, 2023 - Elsevier
In patients with amyotrophic lateral sclerosis (ALS), disease symptoms and pathology
typically spread in a predictable spatiotemporal pattern beginning at a focal site of onset and …
typically spread in a predictable spatiotemporal pattern beginning at a focal site of onset and …
Does wild-type Cu/Zn-superoxide dismutase have pathogenic roles in amyotrophic lateral sclerosis?
Y Furukawa, E Tokuda - Translational Neurodegeneration, 2020 - Springer
Amyotrophic lateral sclerosis (ALS) is characterized by adult-onset progressive
degeneration of upper and lower motor neurons. Increasing numbers of genes are found to …
degeneration of upper and lower motor neurons. Increasing numbers of genes are found to …
Transmission of ALS pathogenesis by the cerebrospinal fluid
PS Mishra, H Boutej, G Soucy, C Bareil… - Acta neuropathologica …, 2020 - Springer
To test the hypothesis that the cerebrospinal fluid (CSF) could provide a spreading route for
pathogenesis of amyotrophic lateral sclerosis (ALS), we have examined the effects of …
pathogenesis of amyotrophic lateral sclerosis (ALS), we have examined the effects of …
Wild-type Cu/Zn-superoxide dismutase is misfolded in cerebrospinal fluid of sporadic amyotrophic lateral sclerosis
E Tokuda, Y Takei, S Ohara, N Fujiwara… - Molecular …, 2019 - Springer
Background A subset of familial forms of amyotrophic lateral sclerosis (ALS) are caused by
mutations in the gene coding Cu/Zn-superoxide dismutase (SOD1). Mutant SOD1 proteins …
mutations in the gene coding Cu/Zn-superoxide dismutase (SOD1). Mutant SOD1 proteins …
Defining novel functions for cerebrospinal fluid in ALS pathophysiology
Despite the considerable progress made towards understanding ALS pathophysiology,
several key features of ALS remain unexplained, from its aetiology to its epidemiological …
several key features of ALS remain unexplained, from its aetiology to its epidemiological …