Thursday, April 19, 2012

New Insight Into the Cellular Defects in Huntington's Disease

Researchers at the Instut Curie in France have recently discovered a new function for the Htt protein concerning Huntington’s disease. Huntington’s disease is a neurogenerative disorder that over a span of time decreases mobility and function, and shortens the lifespan of a person with the disorder. It is caused by a mutant Htt gene and is inherited. Symptoms commonly develop during the mid 30s and 40s, but it may vary between individuals.
In a study done on mice led by Frédéric Saudou, the Htt protein was found to regulate the formation of cilia in the cells. They also found that the cilia in Huntington’s patients and in the mouse model with the disorder were longer and disorganized. Due to these findings, the researchers feel that the longer cilia could be a cause of some symptoms of Huntington’s disease, and plan to continue studies on this idea to test the validity of it. Other researchers, by Scott Zeitlin and Jeh-Ping Liu, at the University of Virginia say that this idea proposed bySaudou is important because it could help determine whether or not therapeutic strategies to normalize the cilia could in turn help alleviate the symptoms of Huntington’s disease in patients with the disorder.

Experimental therapeutics in mouse models of Huntington's disease
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1 comment:

  1. chrismApril 27, 2012 at 9:31 AM

    Symptoms like longer cilia could be a major factor in detecting Huntington's Disease, and possibly finding a way to better treat it. In most cases this degenerative disease is a death sentence, as most people diagnosed die within 20 years of showing visible symptoms. Earlier detection can also be effective in preventing an affected parent from having children, thus saving their offspring from a similar fate.

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