Huntington’s disease research news. In plain language. Written by scientists. For the global HD community.
Figuring out how the mutant huntingtin protein causes damage is the central problem of Huntington's disease research. Now a team of Canadian researchers led by Dr Ray Truant has shown that the protein has an important 'hinge' function, which works less well in cells with the HD mutation. Exciting stuff, but contrary to what you might have read, it doesn't mean we no longer need to study mice!
New research is helping understand how the mutant huntingtin protein moves around the cell. Discovering where huntingtin ends up, and why, could help us understand HD. Now, Canadian researchers have shown that a small piece of the huntingtin protein behaves like an 'address label' for the whole protein. By studying this label and how it affects Huntington's disease symptoms, we may be able to better understand what goes wrong in HD and hopefully generate a disease-modifying therapy.
The human brain is enriched in many different types of fat. Some of these greasy molecules are reduced in HD, and a new study demonstrates that replacing one specific type, called ganglioside GM1, leads to dramatic improvements in the behavior of HD mice.
The mutant huntingtin protein causes Huntington's disease, but not all mutant huntingtin is equally toxic. Exactly where it's found inside cells has a major impact on whether cells can deal with the mutant protein. New research is helping understand the signals that move mutant huntingtin around the cell, provides clues about how to make it less toxic.