Brief Communication

Changes of copper-transporting proteins and ceruloplasmin in the lentiform nuclei in primary adult-onset dystonia

  1. Daniela Berg MD1,
  2. Andreas Weishaupt PhD1,
  3. Michael J Francis DPhil2,
  4. Naoyuki Miura MD3,
  5. Xiao-Li Yang MD4,
  6. Ian D. Goodyer PhD2,
  7. Markus Naumann MD1,
  8. Martin Koltzenburg MD1,
  9. Karlheinz Reiners MD1,
  10. Georg Becker MD1,*

Article first published online: 9 MAY 2001

DOI: 10.1002/1531-8249(200006)47:6<827::AID-ANA20>3.0.CO;2-H

Issue

Annals of Neurology

Annals of Neurology

Volume 47, Issue 6, pages 827–830, June 2000

Additional Information

How to Cite

Berg, D., Weishaupt, A., Francis, M. J., Miura, N., Yang, X.-L., Goodyer, I. D., Naumann, M., Koltzenburg, M., Reiners, K. and Becker, G. (2000), Changes of copper-transporting proteins and ceruloplasmin in the lentiform nuclei in primary adult-onset dystonia. Ann Neurol., 47: 827–830. doi: 10.1002/1531-8249(200006)47:6<827::AID-ANA20>3.0.CO;2-H

Author Information

  1. 1

    Department of Neurology, Bayerische Julius-Maximilians-Universität, Würzburg, Germany

  2. 2

    Human Genetics/Clinical Medicine, Wellcome Trust Centre for Human Genetics, Oxford, UK

  3. 3

    Department of Biochemistry, Hamamatsu University School of Medicine, Hamamatsu, Japan

  4. 4

    Department of Biochemistry, Akita University School of Medicine, Akita, Japan

*Neurologische Klinik und Poliklinik, des Klinikums der Universität Würzburg, Josef-Schneider-Str. 11, 97080 Würzburg, Germany

Publication History

  1. Issue published online: 9 MAY 2001
  2. Article first published online: 9 MAY 2001
  3. Manuscript Accepted: 28 FEB 2000
  4. Manuscript Revised: 21 FEB 2000
  5. Manuscript Received: 5 AUG 1999

Funded by

  • Siemens AG, Erlangen, Germany
  • Schering Deutschland GmbH, Berlin, Germany

SEARCH

SEARCH BY CITATION

ARTICLE TOOLS

Get PDF (6902K)

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. Ann Neurol 2000;47:827–830

Get PDF (6902K)