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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
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. Ann Neurol 2001;49:405–408