[HTML][HTML] Mutant huntingtin in glial cells exacerbates neurological symptoms of Huntington disease mice
J Bradford, JY Shin, M Roberts, CE Wang… - Journal of Biological …, 2010 - ASBMB
Huntington disease (HD) is caused by an expansion of the polyglutamine (polyQ) repeat (>
37Q) in huntingtin (htt), and age of onset is inversely correlated with the length of the polyQ
repeat. Mutant htt with expanded polyQ is ubiquitously expressed in various types of cells,
including glia, but causes selective neurodegeneration. Our recent study demonstrated that
expression of the N-terminal mutant htt with a large polyQ repeat (160Q) in astrocytes is
sufficient to induce neurological symptoms in mice (Bradford, J., Shin, JY, Roberts, M …
37Q) in huntingtin (htt), and age of onset is inversely correlated with the length of the polyQ
repeat. Mutant htt with expanded polyQ is ubiquitously expressed in various types of cells,
including glia, but causes selective neurodegeneration. Our recent study demonstrated that
expression of the N-terminal mutant htt with a large polyQ repeat (160Q) in astrocytes is
sufficient to induce neurological symptoms in mice (Bradford, J., Shin, JY, Roberts, M …
