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

A recent study reported an increase of brain tissue copper content in the l entiform nuclei of patients with primary adult-onset dystonia. In this stud y we analyze copper-metabolizing proteins (Menkes protein, Wilson protein, ceruloplasmin) by Western blot analysis in frozen brain tissue (lentiform n uclei) of 3 patients with primary dystonia. Menkes protein was reduced in a ll patients, while Wilson protein and ceruloplasmin were increased in the 2 patients with focal dystonia and reduced in the patient with generalized d ystonia. Our data provides further evidence for a disturbance of copper met abolism in primary dystonia.