Normal cerebellar glutamate dehydrogenase protein in spinocerebellar degeneration.

Immunochemical analyses (Western blots) of cerebellar homogenates for glutamate dehydrogenase (GDH) from patients with spinocerebellar degeneration and control subjects were conducted. Four patients with autosomal dominant Joseph disease type of spinocerebellar degeneration, one patient with autosomal dominant olivopontocerebellar degeneration and four control subjects were studied. GDH was of the same molecular weight and amount in all patients and control subjects. These data together with normal GDH activity from these same homogenates published previously support the view that GDH is not involved in the pathogenesis of these types of dominantly inherited spinocerebellar degeneration.