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- W1781279124 abstract "Abstract Cirrhotic patients display fasting hypermethioninemia and delayed plasma clearance of methionine. These findings could be explained by an impairment in the main hepatic (transsulfuration) pathway for methionine degradation: methionine → S -adenosylmethionine → homocystine → cystathionine → cyst(e)ine → sulfate and taurine. In this study we quantified by pharmacokinetic methods the degree of methionine intolerance after an oral methionine load. The site of metabolic impairment was investigated by measuring the aforementioned amino acids in the venous blood plasma and urine, plus urinary sulfate excretion. In normal subjects, fasting plasma methionine concentration was 26.1 ± 2.5 μmol/L (mean ± SEM). After an oral load the methionine elimination half-life (t12) was 146 ± 10 min. Urine SO 4 2− excretion averaged 632 ± 92 μmol/h before oral load; this rate increased to 1528 ± 248 μmol/h at 0–6 h and 1881 ± 214 μmol/h at 7–12 h after the load. Intermediates (i.e., homocystine, cystathionine, homoserine, or cystine) did not accumulate in the blood or urine after methionine administration. In cirrhotic patients basal plasma methionine concentration at 65.0 ± 19.4 μmole/L, and t12 at 458 ± 81 min were significantly elevated (p 4 2− excretion, initially 664 ± 84 μmol/h, increased after the load to 809 ± 64 μmol/h at 0–6 h and to 1201 ± 107 μmol/h at 7–12 h, but was significantly less than in normals in both periods (p 4 2− within 24 h, cirrhotic patients excreted only 38%. The clearance of methionine after an oral load is retarded in cirrhosis. The simultaneous delay in SO 4 2− excretion implies a block in the transsulfuration pathway. Because intermediates do not accumulate in plasma or urine during the prolonged hypermethioninemia after oral loading, the block appears to be above the level of homocystine synthesis." @default.
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- W1781279124 date "1981-10-01" @default.
- W1781279124 modified "2023-10-09" @default.
- W1781279124 title "Evidence for impairment of transsulfuration pathway in cirrhosis" @default.
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- W1781279124 doi "https://doi.org/10.1016/0016-5085(81)90489-3" @default.
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