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Transport ofl-cystine in isolated perfused proximal straight tubules

  • Transport Processes, Metabolism and Endocrinology; Kidney, Gastrointestinal Tract, and Exocrine Glands
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Abstract

Unidirectional fluxes ofl-35S-cystine and intracellular35S activity were measured in isolated perfused segments of rabbit proximal straight tubule. The absorptive (lumen-to-both) flux ofl-35S-cysteine showed a tendency toward saturation within the concentration limits imposed by the low solubility of cystine (0.3 mmol·l−1). In contrast, for the bath-to-lumen fluxes, there was a linear relation between the bathing solution concentration ofl-35S-cystine and the rate of35S appearance in the lumen. Nonlinear fitting of both sets of unidirectional flux data gave a maximal cystine transport rate (J max) of 1.45±0.27 (SEM) pmol min−1 mm−1, a Michaelis constant (K m) of 0.20±0.07 mmol·l−1, and an apparent permeability coefficient of 0.27±0.11 pmol min−1 mm−1 (mmol·l−1)−1 (approximately 0.06 μm/s). The35S concentration in the cell exceeded that in the lumen by almost 60-fold during the lumen-to-bath flux, and exceeded the bathing solution concentration by 4.7-fold during the bath-to-lumen flux. Thus cystine was accumulated by the cells across either membrane, but over 77% of the intracellular activity was in the form of cysteine. Although the presence of luminall-lysine or cycloleucine inhibited the absorptive flux of cystine, neither amino acid affected the bath-to-lumen flux.

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References

  1. Barfuss, DW, Schafer JA (1979) Active amino acid absorption by proximal convoluted and proximal straight tubules. Am J Physiol 236:F149-F162

    Google Scholar 

  2. Barfuss DW, Schafer JA (1981) Differences in active and passive glucose transport along the proximal nephron. Am J Physiol 240:F322-F332

    Google Scholar 

  3. Barfuss, DW, Mays JM, Schafer JA (1980) Peritubular uptake and transepithelial transport of glycine in isolated proximal tubules. Am J Physiol 238:F324-F333

    Google Scholar 

  4. Brown RR (1967) Aminoaciduria resulting from cycloleucine administration in man. Science 157:432–434

    Google Scholar 

  5. Busse D, Bartel H, Pohl B (1980) Transport ofl-cystine andl-arginine in brush border vesicles derived from rabbit kidney cortex. Renal Physiol 2:152–153

    Google Scholar 

  6. Craan AG (1981) Cystinuria: the disease and its models. Life Sciences 28:5–22

    Google Scholar 

  7. Craan AG, Bergeron M (1975) Experimental cystinuria: the cycloleucine model. I. Amino acid interactions in renal and intestinal epithelia. Can J Pharmacol 53:1027–1036

    Google Scholar 

  8. Crawhall, JC, Segal S (1967) The intracellular ratio of cysteine and cystine in various tissues. Biochem J 105:891–896

    Google Scholar 

  9. Crawhall JC, Scowen EF, Thompson CJ, Watts RWE (1967) The renal clearance of amino acids in cystinuria. J Clin Invest 46:1162–1171

    Google Scholar 

  10. Cusworth DC, Dent CE (1960) Renal clearance of amino acids in normal adults and patients with amino-aciduria. Biochem J 74:550–561

    Google Scholar 

  11. DeFronzo RA, Thier SO (1978) Renal tubular defect in phosphate and amino acid transport. In: Andreoli TE, Hoffman JF, Fanestil DD (eds) Physiology of membrane of disorders. Plenum, New York, pp 1041–1061

    Google Scholar 

  12. Dent CC, Rose GA (1951) Amino acid metabolism in cystinuria. Quart J Med N S 20:205–219

    Google Scholar 

  13. Evered DF (1967) Species differences in amino acid excretion by mammals. Comp Biochem Physiol 23:163–171

    Google Scholar 

  14. Foreman JW, Hwang S-M, Segal S (1980) Transport interactions of cystine and dibasic amino acids in isolated rat renal tubules. Metabolism 29:53–61

    Google Scholar 

  15. Frimpter GW, Horwith M, Furth E, Fellows RE, Thompson DD (1962) Inulin and endogenous amino acid renal clearance in cystinuria: evidence for tubular secretion. J Clin Invest 41:281–288

    Google Scholar 

  16. Goyer RA, Reynolds JO Jr, Elston RC (1969) Characteristics of amino aciduria resulting from cycloleucine administration in pair fed rats. Proc Soc Exptl Biol Med 130:860–863

    Google Scholar 

  17. Greth WE, Thier SO, Segal S (1973) Cellular accumulation ofl-cystine in rat kidney cortex in vivo. J Clin Invest 52:454–461

    Google Scholar 

  18. McNamara PD, Pepe LM, Segal S (1981) Cystine uptake by rat renal brushborder vesicles. Biochem J 194:443–449

    Google Scholar 

  19. Morin CL, Thompson MW, Jackson SH, Sass-Kortsak H (1971) Biochemical and genetic studies in cystinuria: observations on double heterozygotes of genotype I/II. J Clin Invest 40:1961–1976

    Google Scholar 

  20. Robson EB, Rose GA (1951) The effect of intravenous lysine on renal clearances of cystine, arginine and ornithine in normal subjects, patients with cystinuria and Fanconi syndrome and their relatives. Clin Sci 16:75–93

    Google Scholar 

  21. Rosenberg L, Downing S, Segal S (1962) Competitive inhibition of dibasic amino acid transport in rat kidney. J Biol Chem 237:2265–2270

    Google Scholar 

  22. Schafer JA, Andreoli TE (1979) Perfusion of isolated mammalian renal tubules. In: Giebisch G (ed) Transport across biological membranes, vol IV, Transport organs. Springer, Berlin Heidelberg New York, pp 472–528

    Google Scholar 

  23. Schafer JA, Barfuss DW (1980) Membrane mechanisms for transepithelial amino acid absorption and secretion. Am J Physiol 238:F335-F346

    Google Scholar 

  24. Schafer JA, Troutman SL, Andreoli TE (1974) Volume reabsorption, transepithelial potential differences, and ionic permeability properties in mammalian superficial proxima; straight tubules. J Gen Physiol 64:582–607

    Google Scholar 

  25. Schwartzman L, Blair A, Segal S (1966) A common renal transport system for lysine, ornithine, arginine and cysteine. Biochim Biophys Res Commun 23:220–226

    Google Scholar 

  26. Segal S, Crawhall JC (1967) Transport of cystine by human kidney cortex in vitro. Biochem Med 1:141–150

    Google Scholar 

  27. Segal S, Smith I (1969) Delineation of cystine and cysteine transport systems in rat kidney cortex by developmental patterns. Proc Natl Acad Sci 63:926–933

    Google Scholar 

  28. Segal S, McNamara PD, Pepe LM (1977) Transport interaction of cystine and dibasic amino acids in renal brush border vesicles. Science 197:169–171

    Google Scholar 

  29. Silbernagl S (1973) Some problems ofl-cystine transport in the kidney tubule of rat. Pflügers Arch 343:R46

    Google Scholar 

  30. Silbernagl S, Deetjen P (1972) The tubular reabsorption ofl-cystine andl-cysteine. A common transport system withl-arginine or not? Pflügers Arch 337:227–284

    Google Scholar 

  31. Silbernagl S, Foulkes EC, Deetjen P (1975) Renal transport of amino acids. Rev Physiol Biochem Pharmacol 74:105–167

    Google Scholar 

  32. States B, Segal S (1969) Thin layer chromatographic separation of cystine and the N-ethylmaleimide adducts of cysteine and glutathione. Anal Biochem 27:323–329

    Google Scholar 

  33. Völkl H, Silbernagl S (1982a) Mutual inhibition ofl-cystine/l-cysteine and other neutral amino acids during tubular reabsorption. A microperfusion study in rat kidney. Pflügers Arch 395:190–195

    Google Scholar 

  34. Völkl H, Silbernagl S (1982b), Re-examination of the interplay between dibasic amino acids andl-cystine/l-cysteine during tubular reabsorption. Pflügers Arch 395:196–200

    Google Scholar 

  35. Webber WA, Brown JL, Pitts RF (1961) Interactions of amino acids in renal tubular transport. Am J Physiol 200:380–386

    Google Scholar 

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Schafer, J.A., Watkins, M.L. Transport ofl-cystine in isolated perfused proximal straight tubules. Pflugers Arch. 401, 143–151 (1984). https://doi.org/10.1007/BF00583874

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  • DOI: https://doi.org/10.1007/BF00583874

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