A growing number of neurodegenerative diseases have been found to result from the
expansion of an unstable trinucleotide repeat. Over the past 6 years, researchers have …

Autosomal dominant cerebellar ataxias are hereditary neurodegenerative disorders that are
known as spinocerebellar ataxias (SCA) in genetic nomenclature. In the pregenomic era …

Stress granules (SGs) are membraneless cell compartments formed in response to different
stress stimuli, wherein translation factors, mRNAs, RNA-binding proteins (RBPs) and other …

The ubiquitin-proteasome system targets numerous cellular proteins for degradation. In
addition, modifications by ubiquitin-like proteins as well as proteins containing ubiquitin …

A continuous threat to cell function and viability is the contain components of the ubiquitin-
proteasome degraaccumulation in cells of proteins with highly abnormal dative pathway and …

Huntington's disease (HD) is characterized by the accumulation of a pathogenic protein,
Huntingtin (Htt), that contains an abnormal polyglutamine expansion. Here, we report that a …

At least eight inherited human neurodegenerative diseases are caused by expansion of a
polyglutamine domain within the respective proteins 1, 2. This confers dominant toxicity on …

The huntingtin exon 1 proteins with a polyglutamine repeat in the pathological range (51 or
83 glutamines), but not with a polyglutamine tract in the normal range (20 glutamines), form …

Huntington's disease (HD) is an inherited neurodegenerative disorder caused by
polyglutamine (polyQ) expansions in the huntingtin (Ht) protein. A hallmark of HD is the …

Tandem repeat diseases include the neurodegenerative disorders known as polyglutamine
(polyQ) diseases, caused by CAG repeat expansions in the coding regions of the respective …