| Tyrosine metabolism in cirrhosis. | |
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MedLine Citation:
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PMID: 501206 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The aim of this study was to define the enzyme defect responsible for tyrosinemia in cirrhotic patients. The principal hepatic degradation pathway for tyrosine, tyrosine leads to p-hydroxyphenylpyruvic acid equilibrium homogentisic acid leads to CO2 was studied in 18 cirrhotic patients and eight controls. The classic method employed in elucidation of hereditary tyrosinosis was sued. Metabolic intermediates on the pathway were measured in the basal state, and following oral loading doses (50 mg/kg BW) of tyrosine, PHPA, and homogentisic acid. Cirrhotic patients showed a significant increase (p = 0.005) in fasting plasma tyrosine and in basal PHPA excretion and impaired tolerance to all three metabolites when compared to normals. Fifteen of the 18 cirrhotic patients showed tyrosine intolerance which was not accompanied by change in distal metabolites compared to their basal levels. Nevertheless 13 of the 18 did exhibit intolerance of either PHPA or homogentisic acid. We conclude that in contrast to the single complete defect in hereditary disorders of tyrosine metabolism, cirrhotic patients have partial defects at tyrosine transaminase, PHPA oxidase, and homogentisic acid oxidase, the initial step being rate-limiting. |
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Authors:
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B M Nordlinger; J T Fulenwider; G L Ivey; B A Faraj; F M Ali; M Kutner; J M Henderson; D Rudman |
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Publication Detail:
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Type: Journal Article; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: The Journal of laboratory and clinical medicine Volume: 94 ISSN: 0022-2143 ISO Abbreviation: J. Lab. Clin. Med. Publication Date: 1979 Dec |
Date Detail:
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Created Date: 1980-01-28 Completed Date: 1980-01-28 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0375375 Medline TA: J Lab Clin Med Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 832-40 Citation Subset: AIM; IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Female Hepatic Encephalopathy / complications Homogentisic Acid / blood, urine Humans Liver Cirrhosis / complications, metabolism* Liver Function Tests Male Middle Aged Phenylpyruvic Acids / blood, urine Tyramine / blood Tyrosine / blood, metabolism* |
| Chemical | |
Reg. No./Substance:
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0/Phenylpyruvic Acids; 451-13-8/Homogentisic Acid; 51-67-2/Tyramine; 55520-40-6/Tyrosine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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