Normal glycine transport in cultured diploid fibroblasts from hyperglycinaemic subjects.
The glycine uptake in cultured fibroblasts was studied in three children with non-ketotic hyperglycinaemia, one child with D-glyceric acidaemia and hyperglycinaemia and in six normal children, using an improved assay system giving individual protein correction. It was shown that all hyperglycinaemic children had normal uptake rates and kinetic characteristics similar to the controls.
D000592:Amino Acid Metabolism, Inborn Errors / Q000378:metabolism*; D000621:Aminoisobutyric Acids / Q000494:pharmacology; D001693:Biological Transport, Active / Q000187:drug effects; D002478:Cells, Cultured; D002648:Child; D005347:Fibroblasts / Q000378:metabolism; D005988:Glyceric Acids / Q000097:blood; D005998:Glycine / Q000378:metabolism*; D006801:Humans; D007700:Kinetics; D011392:Proline / Q000494:pharmacology; D014633:Valine / Q000494:pharmacology
D000621:Aminoisobutyric Acids; D005988:Glyceric Acids; C100049:2-aminoisobutyric acid; C017911:2-(methylamino)isobutyric acid; D011392:Proline; D014633:Valine; D005998:Glycine