Recent advances suggest that the response of RNA metabolism to stress has an important
role in the pathophysiology of neurodegenerative diseases, particularly amyotrophic lateral …

Although the past two decades have produced exciting discoveries in the genetics and
pathology of amyotrophic lateral sclerosis (ALS), progress in develo** an effective therapy …

Abstract TAR DNA-binding protein 43 (TDP-43) is a highly conserved nuclear RNA/DNA-
binding protein involved in the regulation of RNA processing. The accumulation of TDP-43 …

Background The disease course of amyotrophic lateral sclerosis (ALS) is rapid and,
because its pathophysiology is unclear, few effective treatments are available. Genetic …

Neuronal cell death in the central nervous system has always been a challenging process to
decipher. In normal physiological conditions, neuronal cell death is restricted in the adult …

TDP-43 is an RNA-binding protein with a crucial nuclear role in splicing, and mislocalises
from the nucleus to the cytoplasm in a range of neurodegenerative disorders. TDP-43 …

Cancer, infectious diseases, and metabolic and hereditary genetic disorders are a global
health burden affecting millions of people, with contemporary treatments offering limited …

In the fatal disease—amyotrophic lateral sclerosis (ALS)—upper (corticospinal) motor
neurons (MNs) and lower somatic MNs, which innervate voluntary muscles, degenerate …

Though motor neurons selectively degenerate in amyotrophic lateral sclerosis, other cell
types are likely involved in this disease. We recently generated rNLS8 mice in which human …

Amyotrophic lateral sclerosis (ALS) is primarily characterized by progressive loss of motor
neurons, although there is marked phenotypic heterogeneity between cases. Typical, or …