Inclusion bodies acquitted

In Huntington's disease, clumps of mutant protein seem to be protective rather than harmful

Maria Anderson(manderson@the-scientist.com)
Oct 13, 2004

Inclusion bodies play a protective, not pathogenic, role in Huntington disease, according to this week's Nature cover study by Steven Finkbeiner, from the Gladstone Institute of Neurological Disease. The paper contributes to an ongoing debate about the role of inclusion bodies—intracellular clumps of mutant huntingtin (Htt) protein—in the pathology of diseases like Huntington's and spinocerebellar ataxia.

Finkbeiner and colleagues at the University of California, San Francisco, used a robotic microscope to track the decline of cultured cells infected with mutant Htt—a line of primary neurons developed in 1998 by Finkbeiner to model Huntington disease. Because the researchers could return to the same cell repeatedly, they were able to track disease progression and closely monitor the factors contributing to a cell's fate.

"It's really the ability to follow a single neuron over its entire lifetime… that helped us piece together the story," Finkbeiner told The Scientist. His group found...

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