Clue to Neurodegenerative Disease Discovered

The differing abilities of cellular machinery to clear a mutated protein from the cell nucleus could provide researchers with clues about treating diseases such as spinal cerebellar ataxia type 1, or SCA-1, and other neurodegenerative diseases, said a scientist at Baylor College of Medicine.

In a report appearing in Nature Cell Biology, David Stenoien, Ph.D., Marilyn Mielke, Ph.D., and Michael Mancini, Ph.D., describe how they monitored nuclear inclusions made of mutant ataxin 1 (which causes SCA-1) in living cells. Using laser-based digital microscopy and fluorescently engineered proteins, the group reports that the inclusions are metabolized at different speeds, and can appear completely fluid. These new observations give scientists clues on how to attack the fatal neurodegenerative disease that usually occurs in adulthood, as well as other neurodegenerations associated with misfolded proteins.

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