Research in Progress

Gene Regulation

Blocking SUMO-1: The End of Early Cell Death?

SUMO-1 is a protein responsible for early cell death in people with HD. It modifies the already mutated huntingtin protein, changing its shape and chemical properties, thus rendering it significantly more toxic to the brain and leading to nerve cell death. (Huntingtin becomes toxic when it disrupts the normal functions that are essential to nerve cell health. When this protein becomes even more toxic, it interferes with normal cell functions to the point of causing the cell to die.)

Researchers at UC Irvine recently blocked the binding of SUMO-1 to mutant huntingtin and found that the huntingtin could not become more toxic. Thus, it could not further disrupt cellular processes. A previous study revealed that chemotherapy drugs called HDAC inhibitors prevented nerve cell damage in fruit flies with mutated huntingtin. The researchers are now finding links between SUMO-1 and HDAC inhibitors in an effort to target the cause rather than the symptoms of HD and delay disease progression.

Click here for the article on HD Blog for more information.

Last Modified: 04/12/2007

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