Amyotrophic lateral sclerosis is a late-onset progressive neurodegenerative disease
affecting motor neurons. The etiology of most ALS cases remains unknown, but 2% of …

Background Heat shock transcription factors (Hsfs) are modular transcription factors
encoded by a large gene family in plants. They bind to the consensus sequence …

The heat-shock transcription factor 1 (HSF1) has an important role in the heat-shock
response in vertebrates by inducing the expression of heat-shock proteins (HSPs) and other …

There is substantial evidence that both inflammation and oxidative damage contribute to the
pathogenesis of motor neuron degeneration in the G93A SOD1 transgenic mouse model of …

Background: Riluzole is currently the only Food and Drug Administration–approved
treatment for ALS, but its effect on survival is modest. Objective: To identify potential …

The discovery of missense mutations in the gene coding for the Cu/Zn superoxide dismutase
1 (SOD1) in subsets of familial cases was rapidly followed by the generation of transgenic …

High threshold for stress-induced activation of the heat shock transcription factor, Hsf1, may
contribute to vulnerability of motor neurons to disease and limit efficacy of agents promoting …

Throughout life, organisms and their cells are subjected to various stressors which they must
respond to adaptively in order to avoid disease and death. Accordingly, cells possess a …

The protein chaperoning and ubiquitin–proteasome systems perform many homeostatic
functions within cells involving protein folding, transport and degradation. Of paramount …

Spinal and bulbar muscular atrophy (SBMA) is a hereditary neurodegenerative disease
characterized by slowly progressive muscle weakness and atrophy of bulbar, facial, and …