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

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Abstract
The primary gene defect in the autosomal dominant disease of Huntington''s chorea [HC], is apparently given expression as a generalized membrane abnormality in peripheral tissues. Several publications claim to be able to detect a difference between HC patients and controls by means of the ESR technique. The ESR spectra of the spin probe 4-maleimido-2,2,6,6-tetramethylpiperidinooxyl (MAL-6) was examined 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 no differences were detected between HC patients and normals. 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.

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