Document Detail

Tyrosine metabolism in cirrhosis.
MedLine Citation:
PMID:  501206     Owner:  NLM     Status:  MEDLINE    
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.
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:
Type:  Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
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:
Created Date:  1980-01-28     Completed Date:  1980-01-28     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0375375     Medline TA:  J Lab Clin Med     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  832-40     Citation Subset:  AIM; IM    
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MeSH Terms
Hepatic Encephalopathy / complications
Homogentisic Acid / blood,  urine
Liver Cirrhosis / complications,  metabolism*
Liver Function Tests
Middle Aged
Phenylpyruvic Acids / blood,  urine
Tyramine / blood
Tyrosine / blood,  metabolism*
Reg. No./Substance:
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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