Changes of copper-transporting proteins and ceruloplasmin in the lentiform nuclei in primary adult-onset dystonia.
A recent study reported an increase of brain tissue copper content in the lentiform nuclei of patients with primary adult-onset dystonia. In this study we analyze copper-metabolizing proteins (Menkes protein, Wilson protein, ceruloplasmin) by Western blot analysis in frozen brain tissue (lentiform nuclei) of 3 patients with primary dystonia. Menkes protein was reduced in all patients, while Wilson protein and ceruloplasmin were increased in the 2 patients with focal dystonia and reduced in the patient with generalized dystonia. Our data provides further evidence for a disturbance of copper metabolism in primary dystonia.
Berg
D
D
Department of Neurology, Bayerische Julius-Maximilians-Universität, Würzburg, Germany.
Weishaupt
A
A
Francis
M J
MJ
Miura
N
N
Yang
X L
XL
Goodyer
I D
ID
Naumann
M
M
Koltzenburg
M
M
Reiners
K
K
Becker
G
G
D000251:Adenosine Triphosphatases / Q000032:analysis / Q000378:metabolism*; D000368:Aged; D002352:Carrier Proteins / Q000032:analysis / Q000378:metabolism*; D027682:Cation Transport Proteins; D002570:Ceruloplasmin / Q000032:analysis / Q000378:metabolism*; D003300:Copper / Q000378:metabolism; D000073840:Copper-Transporting ATPases; D020821:Dystonic Disorders / Q000378:metabolism*; D005260:Female; D005917:Globus Pallidus / Q000737:chemistry / Q000378:metabolism*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011699:Putamen / Q000737:chemistry / Q000378:metabolism*; D011993:Recombinant Fusion Proteins; D012016:Reference Values; D044465:White People
D002352:Carrier Proteins; D027682:Cation Transport Proteins; D011993:Recombinant Fusion Proteins; D003300:Copper; D002570:Ceruloplasmin; D000251:Adenosine Triphosphatases; C081184:ATP7A protein, human; D000073840:Copper-Transporting ATPases