High levels of familial Amyotrophic Lateral Sclerosis (ALS)-linked SOD1 mutants G93A and
G37R were previously shown to mediate disease in mice through an acquired toxic property. …

Huntington's disease (HD) is an inherited, neurodegenerative disorder caused by the expansion
of a glutamine repeat in the N-terminus of the huntingtin protein. To gain insight into the …

Approximately 10% of cases of Alzheimer's disease are familial and associated with autosomal
dominant inheritance of mutations in genes encoding the amyloid precursor protein 1 , …

… Western blot analysis of low molecular weight proteins using the N-terminal MAb, however,
failed to detect a SNAP-25 polypeptide of ∼6 kD, confirming that neither the abnormally …

Huntington's disease (HD) is caused by CAG triplet repeat expansion inIT15which leads to
polyglutamine stretches in the HD protein product, huntingtin. The pathological hallmark of …

An expanded polyglutamine domain in huntingtin underlies the pathogenic events in Huntington
disease (HD), characterized by chorea, dementia and severe weight loss, culminating …

Mutations in superoxide dismutase 1 (SOD1; EC 1.15.1.1 ) are responsible for a proportion
of familial amyotrophic lateral sclerosis (ALS) through acquisition of an as-yet-unidentified …

… Measurements were performed at 9.77 GHz with a Bruker ER 300 spectrometer using a TM
110 cavity and flat cell with microwave power of 20 mW, and a modulation amplitude of 0.5 G…

… (330 aa) aggregated into a high molecular weight polymer within 45 min. There was no …
aa), and httQ67 (135 aa) aggregated into a high molecular weight polymer within 45 min. Fig. 2C …

Dentatorubral and pallidoluysian atrophy (DRPLA) is an autosomal dominant disorder that
clinically overlaps with Huntington's disease (HD) and manifests combinations of chorea, …