Article
Analysis of mutant and total huntingtin expression in Huntington's disease murine models.
Scientific reports - 17 Dec 2020
Fodale Valentina, Pintauro Roberta, Daldin Manuel, Altobelli Roberta, Spiezia Maria Carolina, Bisbocci Monica, Macdonald Douglas, Bresciani Alberto
Abstract excerpt
Huntington's disease (HD) is a monogenetic neurodegenerative disorder that is caused by the expansion of a polyglutamine region within the huntingtin (HTT) protein, but there is still an incomplete understanding of the molecular mechanisms that drive pathology. Expression of the mutant form of HTT is a key aspect of diseased tissues, and the most promising therapeutic approaches aim to lower expanded HTT levels....
Topics
- Animals
- Biomarkers
- Brain
- Disease Models, Animal
- Fibroblasts
- Gene Expression
- Gene Knock-In Techniques
- Humans
- Huntingtin Protein
- Huntington Disease
- Immunoassay
