Electron spin resonance studies of erythrocyte ghost cells in Huntington's chorea.

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.