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Team finds method to reduce accumulation of damaging Huntington's disease protein

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A study appearing in the journal Neuron suggests there may be a new way to change the damaging course of Huntington's disease (HD).

University of California, Irvine (UCI) neurobiologists Leslie Thompson and Joseph Ochaba with the Departments of Neurobiology & Behavior and Psychiatry & Human Behavior and their colleagues from UCI and from Children's Hospital of Philadelphia have shown that reducing the aberrant accumulation of a particular form of the mutant Huntingtin protein corresponds to improvement in symptoms and neuroinflammation in HD mice.

See Also: Test developed to measure effectiveness of treatments for Huntington’s disease

They showed this by targeting and modulating levels of PIAS1—a protein implicated in cancer and other diseases—which they found led to the reduction of the mutant Huntington protein.

The work suggests that changing levels of the PIAS1 protein and targeting this pathway could have a benefit to disease.

There are no current treatments for HD, although Thompson's ongoing work with stem cell-based therapies are showing promise.

Note: Material may have been edited for length and content. For further information, please contact the cited source.

University of California, Irvine   press release


Ochaba J et al. PIAS1 Regulates Mutant Huntingtin Accumulation and Huntington’s Disease-Associated Phenotypes In Vivo. Neuron, Published Online April 14 2016. doi: 10.1016/j.neuron.2016.03.016