Robust, quantitative detection of aggresomes relevant to the study of neurodegenerative disease, liver disease and toxicology.

The ProteoStat®Aggresome detection kit assay is potentially applicable to the identification of small molecules that inhibit aggresome formation as well as immuno-localization studies between aggregated protein cargo and the various proteins implicated in aggresome formation, such as histone deacetylase 6, parkin, ataxin-3, dynein motor complex and ubiquilin-1.

Aggresomes are inclusion bodies that form when the ubiquitin–proteasome machinery is overwhelmed with aggregation-prone proteins. Typically, an aggresome forms in response to some cellular stress, such as hyperthermia, viral infection, or exposure to reactive oxygen species. Aggresomes may provide a cytoprotective function by sequestering the toxic, aggregated proteins and may also facilitate their ultimate elimination from cells by autophagy.Certain cellular inclusion bodies associated with human disease are thought to arise from an aggresomal response including Alzheimer’s disease as it relates to intracellular and extracellular aggregates, Lewy bodies associated with neurons in Parkinson's disease, Mallory bodies associated with liver cells in alcoholic liver disease and Hyaline inclusion bodies associated with astrocytes in amyotrophic lateral sclerosis (ALS).ProteoStat® Aggresome Detection kit provides a rapid, specific and quantitative approach to identify inhibitors relevant to neurodegenerative disease in an authentic cellular context. This fixed-cell assay doesn’t require non-physiological protein mutations or genetically engineered cell lines. ProteoStat® dye has been validated under a wide range of conditions and with small molecule modulators, demonstrating suitability for screening compounds of potential therapeutic value, and optimized for antibody co-localization studies to identify interactions between aggregated protein cargo and the various proteins implicated in autophagy and aggresome formation.