Reduction of Menkes mRNA and copper in leukocytes of patients with primary adult‐onset dystonia | Zendy

Kruse Niels | Zendy; Berg Daniela | Zendy; Francis Michael J. | Zendy; Naumann Markus | Zendy; Rausch WolfDieter | Zendy; Reiners K. | Zendy; Rieckmann Peter | Zendy; Weishaupt Andreas | Zendy; Becker Georg | Zendy

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

Author(s) -

Kruse Niels,

Berg Daniela,

Francis Michael J.,

Naumann Markus,

Rausch WolfDieter,

Reiners K.,

Rieckmann Peter,

Weishaupt Andreas,

Becker Georg

Publication year - 2001

Publication title -

annals of neurology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 4.764

H-Index - 296

eISSN - 1531-8249

pISSN - 0364-5134

DOI - 10.1002/ana.80

Subject(s) - dystonia , basal ganglia , menkes disease , cervical dystonia , blepharospasm , endocrinology , medicine , focal dystonia , basal (medicine) , copper , pathology , biology , central nervous system , chemistry , neuroscience , copper metabolism , organic chemistry , insulin

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. Ann Neurol 2001;49:405–408

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