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Huntington's disease

Flipping a switch on huntingtin

Phosphomimetic mutations at huntingtin (Htt) Ser13 and Ser16 within the conserved N-terminal 17-amino-acid domain profoundly suppresses its toxicity in cell and mouse models of Huntington's disease. New research reveals that cell stress acts as a stimulus for double phosphorylation of endogenous Htt, causing its nuclear translocation, and shows that certain chemicals can target such molecular processes in Huntington's disease cell models.

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Figure 1: A schematic of Htt N17-mediated physiological and pathological pathways in Huntington's disease.

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Correspondence to X William Yang.

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Greiner, E., Yang, X. Flipping a switch on huntingtin. Nat Chem Biol 7, 412–414 (2011). https://doi.org/10.1038/nchembio.604

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