Recombinant Human ATXN3 Protein，His

Ataxin-3 (ATXN3) is a polyglutamine (polyQ) tract-containing protein encoded by the ATXN3 gene, and it has garnered significant attention due to its association with spinocerebellar ataxia type 3 (SCA3), a neurodegenerative disorder characterized by progressive gait and coordination deficits. Research on ATXN3 primarily focuses on understanding its function in cellular processes, as well as the molecular mechanisms underlying its pathogenic aggregation. ATXN3 is known to possess deubiquitinating enzyme activity, which plays a crucial role in protein homeostasis and degradation pathways, thereby influencing the stability and turnover of various cellular proteins. The aggregation of mutant ATXN3, which contains an expanded polyQ region, leads to the formation of toxic inclusions that disrupt cellular function, ultimately contributing to neuronal cell death. Studies have demonstrated that ATXN3 has implications beyond SCA3, as it is involved in other diseases characterized by protein misfolding and aggregation, such as Alzheimer’s and Parkinson’s diseases. Recent advances in recombinant protein techniques have allowed for the production of ATXN3 in vitro, facilitating detailed investigations into its structure-function relationship, aggregation propensity, and interaction with other cellular factors. These studies aim to elucidate the pathways involved in ATXN3-related pathogenesis, paving the way for potential therapeutic strategies targeting the molecular underpinnings of SCA3 and related disorders. Understanding the role of ATXN3 in neurodegeneration may also shed light on the broader aspects of protein aggregation diseases, highlighting its significance in both basic research and clinical contexts.