Several hereditary neurological and neuromuscular diseases are caused by an abnormal
expansion of trinucleotide repeats. To date, there have been 10 of these trinucleotide repeat …

Intrinsically disordered proteins and proteins with intrinsically disordered regions have been
shown to be highly prevalent in disease. Furthermore, disease-causing expansions of the …

Expanded polyglutamine (polyQ) stretches in at least nine unrelated proteins lead to
inherited neuronal dysfunction and degeneration. The expansion size in all diseases …

Huntington's disease (HD) is a fatal neurodegenerative disorder characterized by motor
dysfunction, cognitive deficits and psychosis. HD is caused by mutations in the Huntingtin …

Ataxin-3, the disease protein in Machado-Joseph disease, is known to be proteolytically
modified by various enzymes including two major families of proteases, caspases and …

Proteolytic cleavage has been implicated in the pathogenesis of diverse neurodegenerative
diseases involving abnormal protein accumulation. Polyglutamine diseases are a group of …

Spinocerebellar ataxias are a large group of heterogeneous diseases that all involve
selective neuronal degeneration and accompanied cerebellar ataxia. These diseases can …

At least nine neurodegenerative diseases that are caused by the aggregation induced by
long tracts of glutamine sequences have been identified. One such polyglutamine …

Machado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3, is a rare
neurodegenerative disease. Among all autosomal dominantly inherited ataxias, it is the most …