Electron spin resonance study of membrane protein alterations in erythrocytes in Huntington's disease

Abstract

HUNTINGTON'S disease (HD), a degenerative disease of the central nervous system inherited as an autosomal dominant trait, is characterised clinically by progressive choreiform movements and dementia1. Pathological changes include atrophy, severe cell loss and gliosis in the caudate nucleus and putamen and to a lesser extent the globus pallidus and cerebral cortex2. The aetiology and pathogenesis of this disease are not understood. Although HD is considered a defect of the basal ganglia and cerebral cortex, we report here evidence obtained by electron spin resonance (ESR) techniques (methods reviewed in ref. 3) which suggests HD may have more widespread membrane involvement.