Copper metallothionein in patients with hepatic copper overload.
We studied the Cu-MT present in the hepatic cytosol obtained from 7 patients suffering from conditions associated with hepatic Cu overload (Wilson's disease, biliary atresia, familial cholestatic cirrhosis). Since chromatographic methods appropriate for the isolation of Zn- and Cd-MT were unsuitable for Cu-MT, we developed an indirect procedure for the estimation and resolution of the latter. This procedure involved the preparation of apo-MT and its reconstitution to holo-MT with Zn or Cd. Three predominant isoforms of MT were present in all specimens. Our results indicate that at most 36 +/- 5% of the Cu present in the 10 kDa fraction of cytosol is bound to MT in the liver of patients with hepatic copper overload.
Hunziker
P E
PE
Biochemisches Institut, Universität Zürich, Switzerland.
Sternlieb
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I
D000293:Adolescent; D000328:Adult; D001656:Biliary Atresia / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D003300:Copper / Q000378:metabolism*; D003600:Cytosol / Q000378:metabolism; D005260:Female; D006527:Hepatolenticular Degeneration / Q000378:metabolism; D006801:Humans; D008099:Liver / Q000378:metabolism*; D008105:Liver Cirrhosis, Biliary / Q000378:metabolism; D008297:Male; D008668:Metallothionein / Q000378:metabolism*
C007121:copper thionein; D003300:Copper; D008668:Metallothionein