Courtesy of Steven Finkbeiner
Inclusion bodies play a protective, not pathogenic, role in Huntington disease, according to a recent study by Steven Finkbeiner of the Gladstone Institute of Neurological Disease at the University of California, San Francisco.1 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 such as Huntington and spinocerebellar ataxia.
Finkbeiner and colleagues at UCSF used a robotic microscope to track the decline of cultured cells infected with mutant Htt. Because the researchers could return to the same cell repeatedly, they were able to track disease progression and monitor factors contributing to a cell's fate. They found that cells with multiple inclusion bodies were more likely to survive than those without any. "These clumps, when they form, actually predict better survival, not worse, which is just the opposite of what ...
