mHTT Aggregation/Lowering Assay Service
Huntington's disease (HD) is a devastating neurodegenerative disorder, and mutant huntingtin protein (mHTT) aggregation is a hallmark of its pathogenesis. Our mHTT aggregation/lowering assay service provides a robust and reliable platform for quantifying mHTT aggregation and expression levels. This comprehensive, one-stop solution empowers researchers to gain critical insights into HD mechanisms and accelerate the development of effective therapies. For further information regarding the products and services provided, project-specific consultation, and pricing, please submit an inquiry here.
Introduction
Aggregation of mHTT is a key link in the pathogenesis of HD. The mHTT protein is caused by an abnormal expansion of the CAG trinucleotide repeat sequence, and its N-terminal polyglutamine (polyQ) region is expanded to form an unstable protein structure. The mHTT protein can spontaneously aggregate to form monomers, oligomers, and fibril-rich inclusion bodies (IBs). These aggregates accumulate in cells, leading to cellular dysfunction and neuronal death. The aggregation process is generally divided into primary nucleation, the exponential growth phase, and the stable phase. Primary nucleation is spontaneous, followed by the promotion of aggregation of more mHTT protein through a template-guided mechanism.
Platform for Detection of Huntington Protein
- Ultrasensitive Detection Technology
A novel ELISA-based platform has been developed for the comprehensive quantification of HTT in preclinical models of HD. This platform enables the simultaneous detection and measurement of various HTT species, including full-length, dispersed, and aggregated forms, as well as soluble mHTT monomers and aggregates, offering a more nuanced understanding of HTT pathology.
- Multi-Dimensional Analysis Capabilities
This platform is unique in its ability to simultaneously detect and quantify soluble and aggregated mHTT, as well as intranuclear and extracellular IBs using integrated immunohistochemistry. This comprehensive approach offers unprecedented insights into mHTT pathology and promises to significantly advance HD research.
Fig.1 Quantification of mHTT seeding activity and detection of mHTT fibrils in immunoprecipitate-enriched mouse brain homogenates.2, 3
Application
- This platform plays a vital role in evaluating the efficacy of HTT-lowering therapies, including ASOs, siRNAs, and PROTACs. Accurate measurement of HTT reduction is essential for determining if these treatments can provide meaningful benefit to HD research.
- Furthermore, the platform offers a robust means of monitoring the efficacy of gene therapies. By quantifying transgene expression and the resulting changes in protein levels, it provides critical data for assessing the success of these advanced treatment strategies.
For precise quantification of both soluble and aggregated mHTT levels across all stages of HD drug development, our service offers a unique integration of multiple analytical platforms. We can provide the data you need. Contact our customer service team today for a detailed quote and expert technical support.
References
- Jarosińska, Olga D., and Stefan GD Rüdiger. "Molecular strategies to target protein aggregation in Huntington’s disease." Frontiers in molecular biosciences 8 (2021): 769184.
- Schindler, Franziska, et al. "Small, seeding-competent huntingtin fibrils are prominent aggregate species in brains of zQ175 Huntington’s disease knock-in mice." Frontiers in Neuroscience 15 (2021): 682172.
- Distributed under Open Access license CC BY 4.0, without modification.
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