Abstract:
:The plasma clearance and the liver uptake of intravenously administered (64)Cu were significantly impaired in four patients with Wilson's disease. These defects were unlikely to be simply expressions of the high liver copper concentration as the plasma clearance and hepatic uptake of (64)Cu were normal in four patients with primary biliary cirrhosis, in whom the liver copper concentration was raised to a degree comparable to that in Wilson's disease. The normal liver uptake and plasma clearance of (64)Cu in three patients with other forms of hepatocellular disease suggest that impaired liver cell function does not have a significant effect. The precise nature of the defect in copper transport in Wilson's disease remains to be elucidated; it is possible that delayed uptake of copper by the hepatic lysosomes may account for the toxic effects of the metal.
journal_name
Gutjournal_title
Gutauthors
Smallwood RA,McIlveen B,Rosenoer VM,Sherlock Sdoi
10.1136/gut.12.2.139subject
Has Abstractpub_date
1971-02-01 00:00:00pages
139-44issue
2eissn
0017-5749issn
1468-3288journal_volume
12pub_type
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