Heat shock factor Hsf in nonvertebrate animals and homologous heat shock factor Hsf1 in
vertebrate animals are key transcriptional regulators of the stress protein response. Hsf/Hsf1 …

Abstract Charcot-Marie-Tooth disease (CMT) is the most common inherited neuromuscular
disease and is characterized by considerable clinical and genetic heterogeneity. We …

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative condition in which
motoneurons of the spinal cord and motor cortex die, resulting in progressive paralysis,. This …

Human motoneuron disease is characterized by loss of motor endplates, axonal
degeneration, and cell death of motoneurons. The identification of the underlying gene …

Loss-of-function mutations in the parkin gene, which encodes an E3 ubiquitin ligase, are the
major cause of early-onset Parkinson's disease (PD). Decreases in parkin activity may also …

Over 100 mutants in superoxide dismutase 1 (SOD1) are reported in familial amyotrophic
lateral sclerosis (ALS). However, the precise mechanism by which they are degraded …

Abstract Mutations in the Cu/Zn‐superoxide dismutase (SOD‐1) gene are responsible for a
familial form of amyotrophic lateral sclerosis (fALS). The present study demonstrated …

Mutations in copper-zinc superoxide dismutase (SOD1) have been linked to a subset of
familial amytrophic lateral sclerosis (fALS), a fatal neurodegenerative disease characterized …

The induction of heat shock proteins (Hsps) serves not only as a marker for cellular stress
but also as a promoter of cell survival, which is especially important in the nervous system …

We have investigated the expression of Hsp25, a heat shock protein constitutively
expressed in motoneurons, in amyotrophic lateral sclerosis (ALS) mice that express G93A …