title: Electron spin resonance studies of erythrocyte ghost cells in Huntington's chorea.
abstract: There is accumulated evidence which suggests that the primary gene defect in the autosomal dominant disease of Huntington's Chorea, is given expression as a generalised membrane abnormality in peripheral tissues. Several publications claim to be able to detect a difference between HC patients and controls by means of the electron spin resonance (ESR) technique. We have examined the electron spin resonance spectra of the spin probe 4-maleimido-2,2,6,6-tetramethylpiperidinooxyl (MAL-6) when incorporated into the membranes of erythrocyte ghost cells of 22 patients with HC and 47 controls, in 2 series of controlled, blind studies and we were unable to detect any difference between HC patients and normals. We conclude that the ESR technique with the probe used is not a sufficiently reliable test for accurate differentiation of HC patients from controls and is certainly not suitable as a method for the diagnosis of potential carriers of this disease.
mesh_terms: D004578:Electron Spin Resonance Spectroscopy; D004591:Electrophoresis, Polyacrylamide Gel; D004910:Erythrocyte Membrane / Q000473:pathology*; D004912:Erythrocytes / Q000473:pathology*; D006801:Humans; D006816:Huntington Disease / Q000473:pathology*; D010865:Pilot Projects