Reduction of Menkes mRNA and copper in leukocytes of patients with primary adult-onset dystonia.

Kruse, N; Berg, D; Francis, M J; Naumann, M; Rausch, W D; Reiners, K; Rieckmann, P; Weishaupt, A et al. · Ann Neurol · 2001

case_control · Level III

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Abstract

Studies on postmortem tissue of patients with primary adult-onset dystonia revealed a significant increase in copper levels and a reduction of copper transporting Menkes protein of the lentiform nuclei. Here we demonstrate that patients with idiopathic adult-onset cervical dystonia (n = 14) have reduced Menkes mRNA copies and lower copper levels in leukocytes compared to controls (n = 17; U test, p < 0.05). Changes were less distinct in patients with blepharospasm. Therefore, disturbances of copper metabolism in focal dystonia may not be restricted to the basal ganglia.

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