How huntingtin kills neurons?

The Scientist blog, August 11th, 2010.

Researchers have revealed new clues to how a defective form of the huntingtin protein may cause the deadly changes that lead to Huntington’s disease — by potentially disrupting the process of neurogenesis, thereby decreasing neural progenitor cells.

“[This is] the first study to demonstrate that normal huntingtin has fundamental developmental roles in mitotic spindle function during development and in the process of neurogenesis,” said Mark Mehler, a neurologist at the Albert Einstein College of Medicine who was not involved in the study. Mehler previously found that in Huntington’s, and other neurodegenerative diseases, this kind of defect can lead to neuronal death later in life. In Huntington’s disease, mutations in the huntingtin protein lead to progressively severe psychiatric, cognitive, and motor dysfunction through the death of brain cells.  Read More >