Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
1984-4-4
pubmed:abstractText
The reduction in the conversion of ornithine to proline by fibroblasts from a patient with hyperornithinaemia with hyperammonaemia and homocitrullinuria cannot be explained by a reduced uptake of exogenous ornithine, an altered total intracellular ornithine content, or reduced conversion of gamma-glutamate semialdehyde to proline. However, neither could the postulated defect in mitochondrial ornithine uptake be demonstrated using the digitonin method. Increasing the ornithine concentration in the medium increased the incorporation of 14C label from ornithine into protein in both the patient's and control cells. In the patient's cells the apparent Km for ornithine was ten times that of the controls, although the Vmax values were comparable. This result parallels the clinical response to ornithine supplementation.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
0141-8955
pubmed:author
pubmed:issnType
Print
pubmed:volume
6
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
143-8
pubmed:dateRevised
2007-3-21
pubmed:meshHeading
pubmed:year
1983
pubmed:articleTitle
Studies on the pathway from ornithine to proline in cultured skin fibroblasts with reference to the defect in hyperornithinaemia with hyperammonaemia and homocitrullinuria.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't