Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: chronic neurodegeneration does not induce Hsp27 activation

A Zourlidou, T Gidalevitz, M Kristiansen… - Human molecular …, 2007 - academic.oup.com
A Zourlidou, T Gidalevitz, M Kristiansen, C Landles, B Woodman, DJ Wells, DS Latchman…
Human molecular genetics, 2007academic.oup.com
Huntington's disease (HD) is caused by an expanded polyglutamine tract in the huntingtin
protein. Mitochondrial dysfunction and free radical damage occur in both R6/2 mice and HD
patient brains and might play a role in disease pathogenesis. In cell culture systems, heat-
shock protein 27 (Hsp27), a small molecular chaperone, suppresses mutant huntingtin-
induced reactive oxygen species formation and cell death. To investigate this in vivo, we
conducted an extensive phenotypic characterization of mice arising from a cross between …
Abstract
Huntington's disease (HD) is caused by an expanded polyglutamine tract in the huntingtin protein. Mitochondrial dysfunction and free radical damage occur in both R6/2 mice and HD patient brains and might play a role in disease pathogenesis. In cell culture systems, heat-shock protein 27 (Hsp27), a small molecular chaperone, suppresses mutant huntingtin-induced reactive oxygen species formation and cell death. To investigate this in vivo, we conducted an extensive phenotypic characterization of mice arising from a cross between R6/2 mice and Hsp27 transgenic mice but did not observe an improvement of the R6/2 phenotype. Hsp27 overexpression had no effect in reducing oxidative stress in the R6/2 brain, assessed by measuring striatal aconitase activity and protein carbonylation levels. Native protein gel analysis revealed that transgenic Hsp27 forms active, large oligomeric species in heat-shocked brain lysates, demonstrating that it is efficiently activated upon stress. In contrast, Hsp27 in double transgenic brains exists predominantly as a low molecular weight, inactive species. This suggests that Hsp27, which is otherwise activatable upon heat shock, remains inactive in the R6/2 model of chronic neurodegeneration. Hsp27 transgenics had been previously shown to be protected from acute stresses such as kainate administration, ischemia/reperfusion heart injury and neonatal nerve injury. Our study is the first to suggest a differential modulation of Hsp27 activation in vivo and, importantly, it illustrates the diverse effect of Hsp27 on acute versus chronic models of disease.
Oxford University Press