PerspectiveNEURODEGENERATION

A Glutamine-Rich Trail Leads to Transcription Factors

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Science  21 Jun 2002:
Vol. 296, Issue 5576, pp. 2149-2150
DOI: 10.1126/science.1073845

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Summary

The tragic neurodegenerative disease Huntington's strikes in midlife and is ultimately fatal. Encouraging new results ( Dunah et al.), eloquently explained in a Perspective by Freiman and Tjian, reveal that a glutamine expansion in the mutant huntingtin protein disrupts transcriptional activation by Sp1 and TAF130, leading to loss of expression of genes essential for neuronal survival.