Reduction of Menkes mRNA and copper in leukocytes of patients with primary adult-onset dystonia.
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.
Kruse
N
N
Department of Neurology, Bayerische Julius-Maximilians-Universität, Würzburg, Germany.
Berg
D
D
Francis
M J
MJ
Naumann
M
M
Rausch
W D
WD
Reiners
K
K
Rieckmann
P
P
Weishaupt
A
A
Becker
G
G
D000251:Adenosine Triphosphatases / Q000097:blood*; D000328:Adult; D000368:Aged; D001479:Basal Ganglia / Q000378:metabolism*; D002352:Carrier Proteins / Q000097:blood*; D027682:Cation Transport Proteins; D003300:Copper / Q000097:blood*; D000073840:Copper-Transporting ATPases; D020821:Dystonic Disorders / Q000097:blood*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D012333:RNA, Messenger / Q000097:blood*; D011993:Recombinant Fusion Proteins
D002352:Carrier Proteins; D027682:Cation Transport Proteins; D012333:RNA, Messenger; D011993:Recombinant Fusion Proteins; D003300:Copper; D000251:Adenosine Triphosphatases; C081184:ATP7A protein, human; D000073840:Copper-Transporting ATPases