Huntington disease is a neurological movement disorder involving massive neuronal death in the caudate-putamen region of the brain. Neither preventive nor curative therapy exists for this disease. The implantation of cross-species striatal neural precursor cells into the lesioned striatum of nonhuman primates (baboons) reduced the abnormal movements seen in the disease model. These abnormal mov...

PURPOSE To test the hypothesis that the T2 shortening observed on MR images of the brain in patients with Parkinson and Huntington diseases is due to tissue iron deposition. METHODS Tissue iron and ferritin assays were performed on postmortem putamen and globus pallidus samples from subjects with Huntington and Parkinson disease. The assays were correlated with T2 measurements. Normal samples...

Huntington disease (HD) is caused by a CAG ⋅ CTG expansion in the huntingtin (HTT) gene. While most research has focused on the HTT polyGln-expansion protein, we demonstrate that four additional, novel, homopolymeric expansion proteins (polyAla, polySer, polyLeu, and polyCys) accumulate in HD human brains. These sense and antisense repeat-associated non-ATG (RAN) translation proteins accumulate...