Dicarboxylic amino acid uptake in normal, Friedreich's ataxia, and dicarboxylic aminoaciduria fibroblasts.
Glutamic and aspartic acid uptake was measured in skin fibroblasts from patients with Friedreich's Ataxia, dicarboxylic aminoaciduria, and normal individuals. The results showed no difference in uptake kinetics of either dicarboxylic amino acids between Friedreich's Ataxia and normal cells, but reduced uptake velocities in dicarboxylic aminoaciduria fibroblasts. Friedreich's Ataxia fibroblasts were, however, less calcium-dependent and more magnesium and phosphate-dependent than controls in glucose-free incubation mixture. This difference might be related to some degree of glucose intolerance by Friedreich's Ataxia fibroblasts in culture.
D000596:Amino Acids / Q000494:pharmacology; D000600:Amino Acids, Dicarboxylic / Q000378:metabolism* / Q000652:urine; D001224:Aspartic Acid / Q000378:metabolism; D002478:Cells, Cultured; D002701:Chloramphenicol / Q000494:pharmacology; D005347:Fibroblasts / Q000378:metabolism*; D005621:Friedreich Ataxia / Q000378:metabolism*; D005947:Glucose / Q000494:pharmacology; D005971:Glutamates / Q000378:metabolism; D006801:Humans; D007461:Iodoacetates / Q000494:pharmacology; D011188:Potassium / Q000494:pharmacology; D011190:Potassium Cyanide / Q000494:pharmacology; D012964:Sodium / Q000494:pharmacology; D013439:Sulfhydryl Reagents / Q000494:pharmacology; D013696:Temperature; D013997:Time Factors; D005680:gamma-Aminobutyric Acid / Q000378:metabolism
D000596:Amino Acids; D000600:Amino Acids, Dicarboxylic; D005971:Glutamates; D007461:Iodoacetates; D013439:Sulfhydryl Reagents; D001224:Aspartic Acid; D005680:gamma-Aminobutyric Acid; D002701:Chloramphenicol; D012964:Sodium; D005947:Glucose; D011190:Potassium Cyanide; D011188:Potassium