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The Neuroscientist
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Is Huntington's a Glutamine Storage Disease?

William S. A. Brusilow

Department of Biochemistry and Molecular Biology Wayne State University School of Medicine, Detroit, Michigan

Huntington's disease is a neurological disorder caused by the expansion of a polyglutamine tract in the protein huntingtin. Several other neurological diseases also result from the expansion of polyglutamine regions in different proteins. Despite intense efforts, no definitive biochemical or physiological role for huntingtin has been described, nor has a function been assigned to the polyglutamine region in unaffected individuals. This article presents the hypothesis that polyglutamine expansions within huntingtin and other polyglutamine proteins provide a function in and of themselves. Incorporating multiple glutamine residues into a protein during synthesis, and releasing them during protein turnover, may represent a means of minimizing interruptions in brain levels of glutamine and glutamate during periods of malnutrition. The number and variety of different proteins containing polyglutamine expansions can be interpreted as a series of evolutionary "experiments" toward a nontoxic form for glutamine storage.

Key Words: Polyglutamine proteins • Huntington's disease • Brain glutamine storage • Brain glutamine/glutamate cycle

The Neuroscientist, Vol. 12, No. 4, 300-304 (2006)
DOI: 10.1177/1073858406289639
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