The deubiquitinating enzyme Ataxin-3 (ATXN3) contains a polyglutamine (PolyQ) region, the
expansion of which causes spinocerebellar ataxia type-3 (SCA3). ATXN3 has multiple …

The pathology of spinocerebellar ataxia type 3, also known as Machado‐Joseph disease, is
triggered by aggregation of toxic ataxin‐3 (ATXN3) variants containing expanded …

The Chk1 protein is essential for genome integrity maintenance and cell survival in
eukaryotic cells. After prolonged replication stress, Chk1 can be targeted for proteasomal …

Abstract Spinocerebellar ataxia type 3 (SCA3)/Machado Joseph disease results from
expansion of the polyglutamine domain in ataxin-3 (Atx3). Atx3 is a transcriptional co …

Physiological adaptation to proteotoxic stress in the endoplasmic reticulum (ER) requires
retrotranslocation of misfolded proteins into the cytoplasm for ubiquitination and elimination …

Polyglutamine repeat expansion in ataxin-3 causes neurodegeneration in the most common
dominant ataxia, spinocerebellar ataxia type 3 (SCA3). Since reducing levels of disease …

Branched ubiquitin (Ub) chains make up a significant proportion of Ub polymers in human
cells and are formed when two or more sites on a single Ub molecule are modified with Ub …

Trinucleotide repeat (TNR) expansions are present in a wide range of genes involved in
several neurological disorders, being directly involved in the molecular mechanisms …

Quercetin glycosides, rutin and isoquercitrin, are potent antioxidants that have been found to
possess neuroprotective effect in diseases like Parkinson's and Alzheimer's disease. In the …

The ATPase associated with various cellular activities p97 has a critical function in the
cytoplasmic degradation of proteins misfolded in the ER (endoplasmic reticulum) through a …