Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
13
pubmed:dateCreated
1982-5-12
pubmed:abstractText
To test whether reduced hepatic uroporphyrinogen decarboxylase activity is a specific and intrinsic defect in porphyria cutanea tarda, we measured enzymatic activity in the livers of 17 patients with porphyria cutanea tarda, 12 "normal" control patients without liver disease, and 41 patients with other forms of porphyria, alcoholic liver disease, hemochromatosis, or chronic hepatitis. Enzyme activity in all the patients with porphyria cutanea tarda was lower than in the patients without this disease, except for one patient with alcohol-induced fatty liver. Reduction of hepatic iron stores by phlebotomy did not alter the enzymatic activity in porphyria cutanea tarda. We conclude that reduced hepatic uroporphyrinogen decarboxylase activity is a specific and intrinsic hepatic defect in porphyria cutanea tarda, but modulation of uroporphyrinogen synthesis by extrinsic factors is required for the full biochemical expression of the disease.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Apr
pubmed:issn
0028-4793
pubmed:author
pubmed:issnType
Print
pubmed:day
1
pubmed:volume
306
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
766-9
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed:year
1982
pubmed:articleTitle
Decreased hepatic uroporphyrinogen decarboxylase activity in porphyria cutanea tarda.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S.