title: Changes of copper-transporting proteins and ceruloplasmin in the lentiform nuclei in primary adult-onset dystonia.
abstract: 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.
authors.affiliation: Department of Neurology, Bayerische Julius-Maximilians-Universität, Würzburg, Germany.
authors.forename: D
authors.initials: D
authors.lastname: Berg
authors.forename: A
authors.initials: A
authors.lastname: Weishaupt
authors.forename: M J
authors.initials: MJ
authors.lastname: Francis
authors.forename: N
authors.initials: N
authors.lastname: Miura
authors.forename: X L
authors.initials: XL
authors.lastname: Yang
authors.forename: I D
authors.initials: ID
authors.lastname: Goodyer
authors.forename: M
authors.initials: M
authors.lastname: Naumann
authors.forename: M
authors.initials: M
authors.lastname: Koltzenburg
authors.forename: K
authors.initials: K
authors.lastname: Reiners
authors.forename: G
authors.initials: G
authors.lastname: Becker
mesh_terms: 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
chemical_list: 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