Autophagosome-like vacuole formation in Huntington's disease lymphoblasts

E Nagata, A Sawa, CA Ross, SH Snyder - Neuroreport, 2004 - journals.lww.com
In an effort to clarify cellular abnormalities in Huntington's disease without the confounding
factor of gross degeneration and postmortem alterations associated with studies of the brain,
we have examined HD patient lymphoblasts. We report pronounced vacuole formation in
patients. The vacuoles possess huntingtin remnants and cathepsin B staining, a lysosomal
marker, suggesting autophagy. The number and size of vacuoles parallel the number of
polyglutamine repeats in patients. Treatment with staurosporine, which augments apoptosis …