Reduction of Menkes mRNA and copper in leukocytes of patients with primary adult-onset dystonia.
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.
Medical subject headings
- Adenosine Triphosphatases
- Basal Ganglia
- Carrier Proteins
- Cation Transport Proteins
- Copper
- Dystonic Disorders
- RNA, Messenger
- Recombinant Fusion Proteins