| pubmed-article:1016232 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:1016232 | lifeskim:mentions | umls-concept:C0024660 | lld:lifeskim |
| pubmed-article:1016232 | lifeskim:mentions | umls-concept:C0022192 | lld:lifeskim |
| pubmed-article:1016232 | lifeskim:mentions | umls-concept:C1704259 | lld:lifeskim |
| pubmed-article:1016232 | lifeskim:mentions | umls-concept:C0311402 | lld:lifeskim |
| pubmed-article:1016232 | lifeskim:mentions | umls-concept:C0870883 | lld:lifeskim |
| pubmed-article:1016232 | lifeskim:mentions | umls-concept:C1705987 | lld:lifeskim |
| pubmed-article:1016232 | pubmed:issue | 3 | lld:pubmed |
| pubmed-article:1016232 | pubmed:dateCreated | 1977-3-21 | lld:pubmed |
| pubmed-article:1016232 | pubmed:abstractText | 1. Normal human urine contains small amounts (less than 4 mg/g of creatinine) of 2-ethylhydracrylic acid, formed, we believe, by a previously undisclosed endogenous catabolic pathway for the oxidation of a newly described series of R metabolites of isoleucine. 2. Urinary excretion of 2-ethylhydracrylic acid is variably increased in defects of isoleucine oxidation at distal steps in the catabolic pathway (3-oxoacyl-CoA thiolase deficiency and methylmalonyl-CoA mutase deficiency) and is diminished when proximal steps of the oxidative pathway are blocked as in branched-chain oxo acid decarboxylase deficiency ('maple-syrup-urine' disease). 3. Precursors of R-pathway metabolites [R(-)-2-methylbutyrate and 2-ethylacrylate ] lead to increased 2-ethylhydracrylate excretion in the mammal(rat, rabbit and dog); the corresponding S metabolites [S(+)-2-methylbutyric acid and tiglic acid ], when given in equimolar amounts, have little effect on its excretion, suggesting that little or no interconversion between S and R metabolites occurs in vivo. 4. Studies with 2H-labelled precursors indicate that conversion of R 2-methylbutyrate into 2-ethylhydracrylic acid occurs by a direct pathway (apparently via 2-ethylacrylic acid). 5. The further oxidation of 2-ethylhydracrylic acid to ethylmalonic acid was demonstrated, and may be analogous to S-metabolite oxidation via methyl malonate. 6. Valine metabolites do not interact with the R=isoleucine pathway under the conditions of these experiments in vivo. | lld:pubmed |
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| pubmed-article:1016232 | pubmed:language | eng | lld:pubmed |
| pubmed-article:1016232 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:1016232 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:1016232 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
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| pubmed-article:1016232 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:1016232 | pubmed:month | Dec | lld:pubmed |
| pubmed-article:1016232 | pubmed:issn | 0264-6021 | lld:pubmed |
| pubmed-article:1016232 | pubmed:author | pubmed-author:MamerO AOA | lld:pubmed |
| pubmed-article:1016232 | pubmed:author | pubmed-author:ScriverC RCR | lld:pubmed |
| pubmed-article:1016232 | pubmed:author | pubmed-author:KlassenG AGA | lld:pubmed |
| pubmed-article:1016232 | pubmed:author | pubmed-author:TjoaS SSS | lld:pubmed |
| pubmed-article:1016232 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:1016232 | pubmed:day | 15 | lld:pubmed |
| pubmed-article:1016232 | pubmed:volume | 160 | lld:pubmed |
| pubmed-article:1016232 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:1016232 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:1016232 | pubmed:pagination | 417-26 | lld:pubmed |
| pubmed-article:1016232 | pubmed:dateRevised | 2009-11-18 | lld:pubmed |
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| pubmed-article:1016232 | pubmed:year | 1976 | lld:pubmed |
| pubmed-article:1016232 | pubmed:articleTitle | Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites. | lld:pubmed |
| pubmed-article:1016232 | pubmed:publicationType | Journal Article | lld:pubmed |
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