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July 25, 2004
Huntington's Surprising Effect On Other Cells
Do yourself a favor and read this on the HD Lighthouse website. Dr. Marsha Miller has some excellent comments on this new, and important, study.
The crux of the study...HD affects other cells of the body also, not just the striatum. However, the other's don't die as a result of the defective gene. This gives researchers valuable clues about the disease and hints for potential treatments.
Dr. LaVonne Goodman, with HDDW, was kind enough to send along these comments on this important study:
I believe the Isacson article to be a pivotal one, and so might deserve a second comment from this “contributing editor”.
Huntington’s disease is caused by one gene. But this gene product, the abnormal Huntingtin protein affects many cellular systems. More than twenty such systems are described. And now we learn from Isacson that these adverse effects also occur in brain cells outside the striatum; And in skin cells. But while these cells have similar Huntingtin effect, they don’t get severely damaged and die.
Isacson studied and described three major cellular systems that are affected in all these cells: (1) mitochondria energy problems, (2) decreased neurotrophic factors, and (3) ineffective proteosome systems (which clear defective proteins). All these other surviving cells express the same problems: But they don’t get severely damaged and die.
It is becoming clear that the damage to striatum comes from many different cellular directions. No longer should there be competing arguments about which system is most important. They probably all are.
She goes on to discuss the importance of testing combinations of 'safe' drugs, which is something that HDDW is focused on. (Thankfully.)
Posted by Dave at July 25, 2004 10:32 PM
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