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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
2
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pubmed:dateCreated |
1977-10-31
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pubmed:abstractText |
L-Ornithine-ketoacid-transaminase deficiency was established in cultured fibroblasts obtained from a patient with hyperornithinaemia (mean ornithine level in serum approximately 100 mumol/l) and gyrate atrophy of the choroid and retina. The deficiency was found both the L-ornithine concentrations of 3.0 mM (about twice the KM value) and 12 mM, indicating that the enzymic defect was not due to a decreased affinity for this substrate. The reliability of the colorimetric assay of ornithine-ketoacid-transaminase activity was established radiochemically. Performance of the radiochemical assay revealed the presence of an impurity in the substrate DL-[2-14C]ornithine - HCl being a strong inhibitor of the enzyme. The passage level and the subcultivation time of the fibroblasts did not influence the enzymic activity.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Sep
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pubmed:issn |
0009-8981
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:day |
1
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pubmed:volume |
79
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
371-7
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:890972-Amino Acids,
pubmed-meshheading:890972-Atrophy,
pubmed-meshheading:890972-Cells, Cultured,
pubmed-meshheading:890972-Child,
pubmed-meshheading:890972-Choroid,
pubmed-meshheading:890972-Female,
pubmed-meshheading:890972-Fibroblasts,
pubmed-meshheading:890972-Humans,
pubmed-meshheading:890972-Ornithine,
pubmed-meshheading:890972-Ornithine-Oxo-Acid Transaminase,
pubmed-meshheading:890972-Retina,
pubmed-meshheading:890972-Retinal Diseases,
pubmed-meshheading:890972-Time Factors,
pubmed-meshheading:890972-Transaminases,
pubmed-meshheading:890972-Uveal Diseases
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pubmed:year |
1977
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pubmed:articleTitle |
L-Ornithine-ketoacid-transaminase deficiency in cultured fibroblasts of a patient with hyperornithinaemia and gyrate atrophy of the choroid and retina.
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pubmed:publicationType |
Journal Article,
In Vitro
|