Chorea Huntington

Researchers in Berlin and Düsseldorf have implicated a new gene in the progression of Huntington’s disease in a brain organoid model. The gene may contribute to brain abnormalities much earlier than previously thought. The study was published in “Nature Communications.”

Alzheimer's, Parkinson's and Huntington's diseases have one thing in common: misfolded proteins damage the nerve cells in the brain. Erich Wanker and Anne Ast explained at the Urania Berlin why the protein origami is so important, how the damage occurs and what can be done about it (recording in German).

Proteins are unrivalled in their diversity and complexity, says Erich Wanker, and the slightest of changes can disrupt entire networks of relationships. He wants to understand why, in brain disorders like Huntington’s disease, such disruptions cause the death of neurons – and how this process can be stopped.

Eduardo Silva Ramos has been awarded a fellowship by the Huntington’s Disease Society of America. The MDC researcher intends to further investigate a protein complex that may have a critical influence on a mutation at the root of Huntington’s disease and use this as a starting point to develop new treatment methods.

The origin of this incurable nervous disease lies in minute fibers formed by chains of the huntingtin protein.

Huntington’s disease is a neurodegenerative disease. It is always fatal. The disease is caused by a defect in the Huntingtin gene. To this day, no therapy will put a stop to the insidious disintegration of brain cells. Fortunately, scientists of the Leibniz-Forschungsinstitut für Molekulare Pharmakologie (FMP) in Berlin in collaboration with their peers working at the Max Delbrück Center for Molecular Medicine (MDC) and at the Charité University Hospital discovered a natural mechanism, which not only suppresses the build-up of pathogenic amyloid fibrils but also disaggregates them.

Bettina Purfürst has seen it all. That's an exaggeration, of course, but she has certainly seen more...

Yes, a lot of what they say about proteins is true: they are often promiscuous, and have trouble maintaining long-term attachments... In an experiment or model, two molecules may flirt with each other and look like a couple, but is their interaction really intimate and direct?

While seeking targets to attack Huntington’s disease, an incurable inherited neurodegenerative disorder, neurobiologists of the research group led by Professor Erich Wanker of the Max Delbrück Center for Molecular Medicine in the Helmholtz Association found what they were looking for. Using a filtering strategy borrowed from criminologists, the researchers systematically filtered interaction networks of various biological databases.