Effect of cysteamine on amino acid pools of cultured fibroblasts and the uptake of L-proline |
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Authors: | Beatrice States Stanton Segal |
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Affiliation: | 1. Division of Biochemical Development and Molecular Diseases, The Children''s Hospital of Philadelphia, Philadelphia, PA 19104 USA;2. Department of Medicine, University of Pennsylvania School of Medicine Philadelphia, PA 19104 USA;3. Department of Pediatrics, University of Pennsylvania School of Medicine Philadelphia, PA 19104 USA |
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Abstract: | Cysteamine when incubated with human normal and cystinotic fibroblasts produces significant alterations in cellular amino acid pools with a decrease in proline, glycine, histidine, and methionine as well as branch chain and dibasic amino acids. Serine content was increased while aspartate, glutamate and glutamine were unchanged. The uptake of L-proline by cysteamine treated cells was impaired suggesting that altered uptake of amino acids may be a factor in the cysteamine effect. |
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