Scientists Reveal the First Detailed Image of Huntington’s Disease Fibrils

Researchers have revealed the intricate structure of protein aggregates contributing to Huntington's disease (HD), a genetic disorder characterized by the degeneration of brain cells due to a mutated huntingtin protein. This study, led by Professor Patrick van der Wel at the University of Groningen, combines computational modeling and experimental techniques to unveil the first detailed image of these harmful protein clumps, known as fibrils. Unlike other neurodegenerative diseases, such as Alzheimer’s and Parkinson’s, the structure of huntingtin aggregates had remained obscure until now. The aggregates are composed of a stable core formed by the mutated protein and a dynamic 'fuzzy coat' that interacts with cellular components, leading to cellular dysfunction. The findings indicate that this fuzzy coat may resist interactions that could target these toxic proteins for degradation, complicating potential therapeutic strategies. The insights gained from this research are crucial for understanding the disease's mechanisms and could assist in developing diagnostics and treatments. This project received support from Huntington's disease foundations funded by families and the public, emphasizing the importance of ongoing research in combating this debilitating condition. The study will be published in Nature Communications.

https://instegro.net/scientists-reveal-the-first-detailed-image-of-huntingtons-disease-fibrils/?fsp_sid=80813