Researchers improve motor function and reduce brain shrinkage in animal models of HD by adjusting the levels of a signaling protein.
The Htt protein, which is mutated in Huntington's disease patients, is critical for wiring the brain in early life, a new study reports.
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Researchers work to understand how expanded polyglutamine tracts form the types of supramolecular structures that are presumed to be toxic to neurons.
A new light-based technique for measuring levels of the toxic protein that causes Huntington's disease (HD) has been used to demonstrate that the protein builds up gradually in blood cells.
HD mice crossbred with mice that produced greater levels of PGC-1alpha showed dramatic improvement. Production of misfolded proteins was essentially eliminated and the mice behaved normally. “Degeneration of brain cells is prevented. Neurons don’t die,” said La Spada.