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Assay Method Information

Assay Name:  Clearance of Mutant Huntingtin Protein - Confirmatory screen
Description:  The mutation underlying Huntington's disease is an expansion of a polyglutamine tract in the N-terminus of the protein huntingtin (htt). Under nonpathogenic conditions, this stretch of glutamines range from 2 to 34 repeats, while greater than 37 repeats invariably leads to the disease. In an inducible mouse model of Huntington's disease, we found that abolishing mutant htt expression led to complete recovery of symptomatic mice (Yamamoto et al. 2000). Tightly linked to the symptomatic reversal was a clearance of both the soluble and aggregated forms of the protein. Can the elimination of the accumulated proteins be a means to bring about recovery in HD A fluorescent cell based assay was developed to visualize and analyze aggregation and clearance of mutant huntingtin protein (Yamamoto et al. 2006). This was accomplished by creating a stable cell line that inducibly expressed the N-terminus of huntingtin protein with 103Q repeats fused to monomeric CFP. This cell line permits
Affinity data for this assay
 

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Last update November 1, 2007
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