*nlm.life
			PubMed Journals: J Biol Chem

  Source:		PMID: 9535906


    		J Biol Chem. 1998 Apr 10;273(15):9158-67.
     
			Caspase cleavage of gene products associated
			with triplet expansion disorders generates
			truncated fragments containing the polyglutamine
			tract.

			Wellington CL(1), Ellerby LM, Hackam AS,
			Margolis RL, Trifiro MA, Singaraja R, McCutcheon
			K, Salvesen GS, Propp SS, Bromm M, Rowland
			KJ, Zhang T, Rasper D, Roy S, Thornberry
			N, Pinsky L, Kakizuka A, Ross CA, Nicholson
			DW, Bredesen DE, Hayden MR.

			Author Information
			(1) Centre for Molecular Medicine and Therapeutics
			and Department of Medical Genetics,
			University of British Columbia, Vancouver,
			British Columbia V6T 1Z4, Canada.

			The neurodegenerative diseases Huntington
			disease, dentatorubropallidoluysian atrophy,
			spinocerebellar atrophy type 3, and spinal
			bulbar muscular atrophy are caused by expansion
			of a polyglutamine tract within their respective
			gene products. There is increasing evidence
			that generation of truncated proteins containing
			an expanded polyglutamine tract may be a
			key step in the pathogenesis of these disorders.
			We now report that, similar to huntingtin,
			atrophin-1, ataxin-3, and the androgen receptor
			are cleaved in apoptotic extracts. Furthermore,
			each of these proteins is cleaved by one
			or more purified caspases, cysteine proteases
			involved in apoptotic death. The CAG length
			does not modulate susceptibility to cleavage
			of any of the full-length proteins. Our
			results suggest that by generation of truncated
			polyglutamine-containing proteins, caspase
			cleavage may represent a common step in
			the pathogenesis of each of these neurodegenerative
			diseases.

			PMID: 9535906 [Indexed for MEDLINE]

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