'Intrabody' can mop up mutant protein in Huntington's dis...(makes the proteins clump together inside...),Health
makes the proteins clump together inside brain cells.
Li says scientists who work on Huntington's have been studying where inside the cell the clumps have their toxic effect: brain cells' nuclei or in their axons and dendrites.
"Our goal here was to create a tool that could distinguish between the accumulation of mutant proteins in the nucleus and the cytoplasm," he says. "The intrabody binds huntingtin proteins with expanded poly-glutamine regions and it only works in the cytoplasm, not the nucleus."
Li and his colleagues showed that cultured cells that make both the intrabody and mutant huntingtin are able to get rid of the mutant protein faster and have fewer clumps of huntingtin.
Even though the intrabody only travels within the cytoplasm, it still alleviated the motor problems of mice that make mutant huntingtin when injected into the striatum, the scientists found. The striatum is the part of the brain most affected by Huntington's disease.
Li says finding an antibody that prefers to bind mutant, aggregated protein could also prove useful in the study of other neurodegenerative disorders, such as Alzheimer's disease or Creutzfeldt-Jakob disease.
"Several neurodegenerative diseases appear to involve defects in protein folding and metabolism, leading to the accumulation of protein aggregates inside cells," he says. "Our study suggests a strategy for dissecting the harmful effects of these protein aggregates in other diseases."
The clumps of mutant huntingtin are also seen in the neuronal nucleus and cytoplasm in a recently established transgenic monkey model of Huntington's, which was reported by Emory University researchers at http://whsc.emory.edu/press_releases2.cfm?announcement_id_seq=14304,
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| Contact: Holly Korschun hkorsch@emory.edu 404-727-3990 Emory University Source:Eurekalert |
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