Glycine transport by cultured skin fibroblasts from a patient with isolated hyperglycinuria.
Glycine transport is studied in cultured skin fibroblasts from a patient with isolated hyperglycinuria and from five normal subjects. Fibroblasts from the patient take up glycine less well than do cell lines from controls. Kinetic studies are consistent with a single transport system in the patient's and controls' cell lines. Vmax value in the hyperglycinuric lines is normal, but the apparent affinity is always reduced as opposed to those of four different control lines separately tested. Statistical analysis shows significant difference between Km values.
D000592:Amino Acid Metabolism, Inborn Errors / Q000378:metabolism*; D001692:Biological Transport; D002478:Cells, Cultured; D002648:Child; D005347:Fibroblasts / Q000378:metabolism*; D005998:Glycine / Q000378:metabolism* / Q000652:urine*; D006801:Humans; D007700:Kinetics; D008297:Male; D012867:Skin
D005998:Glycine