Cell Biology

Tagging Troublesome Trash

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Science  10 Apr 2009:
Vol. 324, Issue 5924, pp. 149
DOI: 10.1126/science.324.5924.149c

Huntington's disease is caused by the accumulation of a mutant form of the protein huntingtin, which folds improperly, leading to neurodegeneration and death. Autophagy provides a means by which cells can degrade aberrant cytosolic proteins within lysosomes. Jeong et al. wondered if the damage caused by mutant huntingtin could be prevented by promoting the clearance of the misfolded protein from affected neurons. In animal and cellular models of Huntington's disease, they found that increasing acetylation of the pathological form of huntingtin enhanced its trafficking via autophagy into lysosomes and its subsequent degradation. Blocking acetylation promoted neurodegeneration in cultured neurons and in mutant mice. Thus, this posttranslational modification might be exploited therapeutically if some means can be devised for the specific acetylation of pathological aggregates of huntingtin. — SMH

Cell 137, 10.1016/j.cell.2009.03.018 (2009).

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