Neuromuscular junction dysfunction in amyotrophic lateral sclerosis

S Verma, S Khurana, A Vats, B Sahu, NK Ganguly… - Molecular …, 2022 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by
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 …

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 …

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 …

[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) …

[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 …

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 …

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 …

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 …

Defining novel functions for cerebrospinal fluid in ALS pathophysiology

KC Ng Kee Kwong, AR Mehta, M Nedergaard… - Acta Neuropathologica …, 2020 - Springer
Despite the considerable progress made towards understanding ALS pathophysiology,
several key features of ALS remain unexplained, from its aetiology to its epidemiological …