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Use of Human Genetic Variation to Study Membrane Transport of Amino Acids in Kidney

Use of Human Genetic Variation to Study Membrane Transport of Amino Acids in Kidney Abstract THE subject of this paper is amino acid transport in human kidney. My interest in this topic began with a study of familial hyperprolinemia1 which Irwin Schafer and I carried out with Mary Efron. Mary then pursued her own interests in the imino acids, and published new and important information on their metabolism in man before she died.2-4 I chose to investigate membrane transport of amino acids because it seemed an interesting challenge, initiated as it was in the supercharged environment which surrounded anything with which Mary was involved. This paper reviews briefly what we are learning about transport of free amino acids from patients with hereditary aminoacidopathies. Dent5 once said that the two-dimensional partition chromatogram of amino acids in normal human urine became so familiar to the habitual observer that deviation from the normal pattern was instantly recognized. So it was with the first chromatogram which References 1. Schafer, I.A.; Scriver, C.R.; and Efron, M.L.: Familial Hyperprolinemia, Cerebral Dysfunction and Renal Anomalies Occurring in a Family With Hereditary Nephritis and Deafness , New Eng J Med 267:51-60 ( (July) ) 1962.Crossref 2. Efron, M.L.: Familial Hyperprolinemia: Report of a Second Case Associated With Renal Malformations, Hereditary Hematuria and Mild Mental Retardation, With Demonstration of an Enzyme Defect , New Eng J Med 272:1243-1254 ( (June) ) 1965.Crossref 3. Efron, M.L.; Bixby, E.M.; and Pryles, C.V.: Hydroxyprolinemia: II. A Rare Metabolic Disease Due to a Deficiency of the Enzyme "Hydroxyproline Oxidase," New Eng J Med 272:1299-1309 ( (June) ) 1965.Crossref 4. Efron, M.: " Disorders of Proline and Hydroxyproline Metabolism ," in Stanbury, J.B.; Wyngaarden, J.B.; and Fredrickson, D.S. (eds.): The Metabolic Basis of Inherited Disease , New York: McGraw-Hill Co., Inc., 1966, pp 376-392. 5. Dent, C.E.: Clinical Applications of Amino Acid Chromatography , Scand J Clin Lab Invest 10 ( (suppl 31) ):122-127 (March) 1957. 6. Woolf, L.I., and Norman, A.P.: The Urinary Excretion of Amino Acids and Sugars in Early Infancy , J Pediat 50:271-295 ( (March) ) 1957.Crossref 7. Scriver, C.R.; Schafer, I.A.; and Efron, M.L.: New Renal Tubular Amino Acid Transport System and a New Hereditary Disorder of Amino Acid Metabolism , Nature 192:672-673 ( (Nov) ) 1961.Crossref 8. Scriver, C.R.: " Amino Acid Transport in Mammalian Kidney ," in W. L. Nyhan (ed.), Amino Acid Metabolism and Genetic Variation , New York: McGraw-Hill Book Co., Inc., 1967, pp 327-340. 9. Leaf, A.: " The Syndrome of Osteomalacia, Renal Glucosuria, Aminoaciduria, and Increased Phosphorus Clearance (The Fanconi Syndrome) ," in Stanbury, J.B.; Wyngaarden, D.S.; and Fredrickson, J.B. (eds.): The Metabolic Basis of Inherited Disease , ed 2, New York: McGraw-Hill Book Co., Inc., 1966, pp 1205-1220. 10. Efron, M.L.: Aminoaciduria , New Eng J Med 272:1058-1107 ( (May 20) ) 1965.Crossref 11. Scriver, C.R.: " Hereditary Aminoaciduria ," in Bearn, A., and Steinberg, A.G. (eds.): Progress in Medical Genetics , New York: Grune & Stratton, Inc., 1962, vol 2, pp 83-186. 12. Rosenberg, L.E.: " Genetic Heterogeneity in Cystinuria ," in W. L. Nyhan (ed.), Amino Acid Metabolism and Genetic Variation , New York: McGraw-Hill Book Co., Inc., 1967, pp 341-349. 13. Dent, C.E., and Walshe, J.M.: Amino Acid Metabolism , Brit Med Bull 10:249-250 (No. (3) ) 1954. 14. Cusworth, D.C., and Dent, C.E.: Renal Clearances of Amino Acids in Normal Adults and in Patients With Aminoaciduria , Biochem J 74:550-561 ( (March) ) 1960. 15. O'Brien, D., and Butterfield, L.J.: Further Studies on Renal Tubular Conservation of Free Amino Acids in Early Infancy , Arch Dis Child 38: 437-442 ( (Oct) ) 1963.Crossref 16. Brodehl, J., et al: The Renal Transport of Free Amino Acids in Infancy and Childhood, Pediatrics, to be published. 17. Scriver, C.R., and Davies, E.: Endogenous Renal Clearance Rates of Free Amino Acids in Pre-pubertal Children , Pediatrics 36:592-598 1965. 18. Pitts, R.F.: A Renal Reabsorptive Mechanism in the Dog Common to Glycine and Creatine , Amer J Physiol 140:156-161 ( (Nov) ) 1943. 19. Scriver, C.R.; Efron, M.L.; and Schafer, I.A.: Renal Tubular Transport of Proline, Hydroxyproline and Glycine in Health and in Familial Hyperprolinemia , J Clin Invest 43:374-385 1964.Crossref 20. Scriver, C.R., and Goldman, H.: Renal Tubular Transport of Proline, Hydroxyproline and Glycine: II. Hydroxy-L-Proline as Substrate and as Inhibitor In-Vivo , J Clin Invest 45:1357-1363 ( (Aug) ) 1966.Crossref 21. Scriver, C.R., and Wilson, O.H.: Possible Location for a Common Gene Product in Membrane Transport of Imino Acids and Glycine , Nature 202:92-93 ( (April) ) 1964.Crossref 22. Wilson, O.H., and Scriver, C.R.: Specificity of Transport of Neutral and Basic Amino Acids in Rat Kidney , Amer J Physiol 213:185-190 1967. 23. Robson, E.B., and Rose, G.A.: The Effect of Intravenous Lysine on the Renal Clearances of Cystine, Arginine and Ornithine in Normal Subjects, in Patients With Cystinuria and Fanconi Syndrome and in Their Relatives , Clin Sci 16: 75-93 ( (Feb) ) 1957. 24. Rosenberg, L.E.; Downing, S.J.; and Segal, S.: Competitive Inhibition of Dibasic Amino Acid Transport in Rat Kidney , J Biol Chem 237:2265-2270 ( (July) ) 1962. 25. Segal, S., et al: Dibasic Amino Acid Transport in Rat-Kidney Cortex Slices , Biochim Biophys Acta 135:127-135 ( (Feb 1) ) 1967.Crossref 26. Webber, W.A.; Brown, J.L.; and Pitts, R.F.: Interactions of Amino Acids During Renal Tubular Transport , Amer J Physiol 200:380-386 ( (Feb) ) 1961. 27. Webber, W.A.: Characteristics of Acidic Amino Acid Transport in Mammalian Kidney , Canad J Biochem Physiol 41:131-137 ( (Jan) ) 1963.Crossref 28. Scriver, C.R.; Pueschel, S.; and Davies, E.: Hyper-β-alaninemia Associated With β-aminoaciduria and y-Aminobutyricaciduria, Somnolence and Seizures , New Eng J Med 274:636-643 1966.Crossref 29. Gilbert, J.B., et al: The Increase in Urinary Taurine After Intraperitoneal Administration of Amino Acids to the Mouse , J Biol Chem 235:1055-1060 ( (April) ) 1960. 30. Goldman, H., and Scriver, C.: A Transport System in Mammalian Kidney With Preference for β-amino Compounds, abstracted , Ped Res 1: 212-213, 1967.Crossref 31. Knox, W.E.: " Cystinuria ," in Stanbury, J. B.; Wyngaarden, J.B.; and Fredrickson, D.S. (eds.): The Metabolic Basis of Inherited Disease , ed 2, New York: McGraw-Hill Book Co., Inc., 1966, pp 1262-1282. 32. Baron, D.N., et al: Hereditary Pellagra-like Skin Rash With Temporary Cerebellar Ataxia, Constant Renal Aminoaciduria and Other Bizarre Biochemical Features , Lancet 2:421-428 ( (Sept 1) ) 1956.Crossref 33. Jepson, J.B.: " Hartnup Disease ," in Stanbury, J.B.; Wyngaarden, J.B.; and Fredrickson, D.S. (eds.): The Metabolic Basis of Inherited Disease , ed 2, New York: McGraw-Hill Book Co., Inc., 1966, pp 1283-1299. 34. Milne, M.D., et al: The Metabolic Disorder in Hartnup Disease , Quart J Med 29:407-421 ( (July) ) 1960. 35. Scriver, C.R.: Hartnup Disease: A Genetic Modification of Intestinal and Renal Transport of Certain Neutral Alpha-Amino Acids , New Eng J Med 273:530-532 ( (Sept) ) 1965.Crossref 36. Scriver, C.R., and Wilson, O.H.: Amino Acid Transport in Human Kidney: Evidence for Genetic Control of Two Types , Science 155:1428-1430 ( (March) ) 1967.Crossref 37. Whelan, D.T., and Scriver, C.R.: Cystathioninuria and Renal Imino-Glycinuria in a Pedigree: A Perspective on Counselling , New Eng J Med 278: 924-927 ( (April) ) 1968.Crossref 38. Tada, K., et al: Prolinuria: A New Renal Tubular Defect in Transport of Proline and Glycine , J Exp Med 87:133-143 ( (Nov 25) ) 1965. 39. Goodman, S.I.; McIntyre, C.A.; and O'Brien, D.: Impaired Intestinal Transport of Proline in a Patient With Familial Iminoaciduria , J Pediat 71: 246-249 ( (Aug) ) 1967.Crossref 40. Rosenberg, L.E.; Devant, J.E.; and Elsas, L.J.: Familial Iminoglycinuria: An Inborn Error of Renal Tubular Transport , New Eng J Med 278: 1407-1413 ( (June) ) 1968.Crossref 41. Scriver, C.R.: Renal Tubular Transport of Proline, Hydroxyproline and Glycine: III. Genetic Basis for More Than One Mode of Uptake in Human Kidney , J Clin Invest 47:823-835 ( (April) ) 1968.Crossref 42. Doolan, P.D., et al: Renal Clearance of Lysine in Cystinuria , Amer J Med 23:416-425 ( (Sept) ) 1957.Crossref 43. Frimpter, G.E., et al: Inulin and Endogenous Amino Acid Renal Clearances in Cystinuria: Evidence for Tubular Secretion , J Clin Invest 41:281-288 ( (Feb) ) 1962.Crossref 44. Crawhall, J.C., et al: The Renal Clearance of Amino Acids in Cystinuria , J Clin Invest 43:1162-1171 ( (July) ) 1967.Crossref 45. Rosenberg, L.E.; Albrecht, I.; and Segal, S.: Lysine Transport in Human Kidney: Evidence for Two Systems , Science 155:1426-1428 ( (March) ) 1967.Crossref 46. Scriver, C.R., and Mohyuddin, F.: Amino Acid Transport in Kidney: Heterogeneity of AIB Uptake , J Biol Chem 243:3207-3213 ( (June) ) 1968. 47. Rosenberg, L.E.: Cystinuria: Genetic Heterogeneity and Allelism , Science 154:1341-1343 ( (Dec) ) 1966.Crossref 48. Segal, S., and Crawhall, J.C.: Characteristics of Cystine and Cysteine Transport in Rat Kidney Cortex Slices , Proc Nat Acad Sci 59:231-237 ( (Jan) ) 1968.Crossref 49. Schwartzman, L.; Blair, A,; and Segal, S.: Exchange Diffusion of Dibasic Amino Acids in Rat Kidney Cortex Slices , Biochim Biophys Acta 135: 120-136 ( (Feb 1) ) 1967.Crossref 50. Brodehl, J.; Gellisen, K.; and Kowalewski, S.: Isolierter Defekt der tubulären Cystin-Rück resorption in einer Familie mit idiopathischem Hypoparathyroidismus , Klin Wschr 45:38-40 ( (Jan 1) ) 1967.Crossref 51. Whelan, D.T., and Scriver, C.R.: Hyperdibasicaminoaciduria: An Inherited Disorder of Amino Acid Transport , Ped Res 2:523-532 ( (Nov) ) 1968.Crossref 52. Kekomäki, M., et al: Familial Protein Intolerance With Deficient Transport of Basic Amino Acids , Acta Paed Scand 56:617-630 ( (Nov) ) 1967.Crossref 53. Harris, H., et al: Pattern of Amino Acid Excretion in Cystinuria , Ann Hum Genet 19:195-208 ( (Feb) ) 1955.Crossref 54. Christensen, H.N.: Reactive Sites and Biological Transport , Advances Protein Chem 15:239-314, 1960. 55. Christensen, H.N.: Some Transport Lessons Taught by the Organic Solute , Perspect Biol Med 10:471-494 (No. (3) ) 1967. 56. Kepes, A.: " The Place of Permeases in Cellular Organization ," in J. F. Hoffman (ed.), The Cellular Functions of Membrane Transport , Englewood Cliffs, NJ: Prentice-Hall, Inc., 1964, pp 155-169. 57. Efron, M.L.: Treatment of Hydroxyprolinemia and Hyperprolinemia , Amer J Dis Child 113:166-169 ( (Jan) ) 1967. 58. Scriver, C.R.: Membrane Transport in Disorders of Imino Acid Metabolism , Amer J Dis Child 113:170-173 ( (Jan) ) 1967. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Use of Human Genetic Variation to Study Membrane Transport of Amino Acids in Kidney

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American Medical Association
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Copyright © 1969 American Medical Association. All Rights Reserved.
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0002-922X
DOI
10.1001/archpedi.1969.02100030006002
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Abstract

Abstract THE subject of this paper is amino acid transport in human kidney. My interest in this topic began with a study of familial hyperprolinemia1 which Irwin Schafer and I carried out with Mary Efron. Mary then pursued her own interests in the imino acids, and published new and important information on their metabolism in man before she died.2-4 I chose to investigate membrane transport of amino acids because it seemed an interesting challenge, initiated as it was in the supercharged environment which surrounded anything with which Mary was involved. This paper reviews briefly what we are learning about transport of free amino acids from patients with hereditary aminoacidopathies. Dent5 once said that the two-dimensional partition chromatogram of amino acids in normal human urine became so familiar to the habitual observer that deviation from the normal pattern was instantly recognized. So it was with the first chromatogram which References 1. Schafer, I.A.; Scriver, C.R.; and Efron, M.L.: Familial Hyperprolinemia, Cerebral Dysfunction and Renal Anomalies Occurring in a Family With Hereditary Nephritis and Deafness , New Eng J Med 267:51-60 ( (July) ) 1962.Crossref 2. Efron, M.L.: Familial Hyperprolinemia: Report of a Second Case Associated With Renal Malformations, Hereditary Hematuria and Mild Mental Retardation, With Demonstration of an Enzyme Defect , New Eng J Med 272:1243-1254 ( (June) ) 1965.Crossref 3. Efron, M.L.; Bixby, E.M.; and Pryles, C.V.: Hydroxyprolinemia: II. A Rare Metabolic Disease Due to a Deficiency of the Enzyme "Hydroxyproline Oxidase," New Eng J Med 272:1299-1309 ( (June) ) 1965.Crossref 4. Efron, M.: " Disorders of Proline and Hydroxyproline Metabolism ," in Stanbury, J.B.; Wyngaarden, J.B.; and Fredrickson, D.S. (eds.): The Metabolic Basis of Inherited Disease , New York: McGraw-Hill Co., Inc., 1966, pp 376-392. 5. Dent, C.E.: Clinical Applications of Amino Acid Chromatography , Scand J Clin Lab Invest 10 ( (suppl 31) ):122-127 (March) 1957. 6. Woolf, L.I., and Norman, A.P.: The Urinary Excretion of Amino Acids and Sugars in Early Infancy , J Pediat 50:271-295 ( (March) ) 1957.Crossref 7. Scriver, C.R.; Schafer, I.A.; and Efron, M.L.: New Renal Tubular Amino Acid Transport System and a New Hereditary Disorder of Amino Acid Metabolism , Nature 192:672-673 ( (Nov) ) 1961.Crossref 8. Scriver, C.R.: " Amino Acid Transport in Mammalian Kidney ," in W. L. Nyhan (ed.), Amino Acid Metabolism and Genetic Variation , New York: McGraw-Hill Book Co., Inc., 1967, pp 327-340. 9. Leaf, A.: " The Syndrome of Osteomalacia, Renal Glucosuria, Aminoaciduria, and Increased Phosphorus Clearance (The Fanconi Syndrome) ," in Stanbury, J.B.; Wyngaarden, D.S.; and Fredrickson, J.B. (eds.): The Metabolic Basis of Inherited Disease , ed 2, New York: McGraw-Hill Book Co., Inc., 1966, pp 1205-1220. 10. Efron, M.L.: Aminoaciduria , New Eng J Med 272:1058-1107 ( (May 20) ) 1965.Crossref 11. Scriver, C.R.: " Hereditary Aminoaciduria ," in Bearn, A., and Steinberg, A.G. (eds.): Progress in Medical Genetics , New York: Grune & Stratton, Inc., 1962, vol 2, pp 83-186. 12. Rosenberg, L.E.: " Genetic Heterogeneity in Cystinuria ," in W. L. Nyhan (ed.), Amino Acid Metabolism and Genetic Variation , New York: McGraw-Hill Book Co., Inc., 1967, pp 341-349. 13. Dent, C.E., and Walshe, J.M.: Amino Acid Metabolism , Brit Med Bull 10:249-250 (No. (3) ) 1954. 14. Cusworth, D.C., and Dent, C.E.: Renal Clearances of Amino Acids in Normal Adults and in Patients With Aminoaciduria , Biochem J 74:550-561 ( (March) ) 1960. 15. O'Brien, D., and Butterfield, L.J.: Further Studies on Renal Tubular Conservation of Free Amino Acids in Early Infancy , Arch Dis Child 38: 437-442 ( (Oct) ) 1963.Crossref 16. Brodehl, J., et al: The Renal Transport of Free Amino Acids in Infancy and Childhood, Pediatrics, to be published. 17. Scriver, C.R., and Davies, E.: Endogenous Renal Clearance Rates of Free Amino Acids in Pre-pubertal Children , Pediatrics 36:592-598 1965. 18. Pitts, R.F.: A Renal Reabsorptive Mechanism in the Dog Common to Glycine and Creatine , Amer J Physiol 140:156-161 ( (Nov) ) 1943. 19. Scriver, C.R.; Efron, M.L.; and Schafer, I.A.: Renal Tubular Transport of Proline, Hydroxyproline and Glycine in Health and in Familial Hyperprolinemia , J Clin Invest 43:374-385 1964.Crossref 20. Scriver, C.R., and Goldman, H.: Renal Tubular Transport of Proline, Hydroxyproline and Glycine: II. Hydroxy-L-Proline as Substrate and as Inhibitor In-Vivo , J Clin Invest 45:1357-1363 ( (Aug) ) 1966.Crossref 21. Scriver, C.R., and Wilson, O.H.: Possible Location for a Common Gene Product in Membrane Transport of Imino Acids and Glycine , Nature 202:92-93 ( (April) ) 1964.Crossref 22. Wilson, O.H., and Scriver, C.R.: Specificity of Transport of Neutral and Basic Amino Acids in Rat Kidney , Amer J Physiol 213:185-190 1967. 23. Robson, E.B., and Rose, G.A.: The Effect of Intravenous Lysine on the Renal Clearances of Cystine, Arginine and Ornithine in Normal Subjects, in Patients With Cystinuria and Fanconi Syndrome and in Their Relatives , Clin Sci 16: 75-93 ( (Feb) ) 1957. 24. Rosenberg, L.E.; Downing, S.J.; and Segal, S.: Competitive Inhibition of Dibasic Amino Acid Transport in Rat Kidney , J Biol Chem 237:2265-2270 ( (July) ) 1962. 25. Segal, S., et al: Dibasic Amino Acid Transport in Rat-Kidney Cortex Slices , Biochim Biophys Acta 135:127-135 ( (Feb 1) ) 1967.Crossref 26. Webber, W.A.; Brown, J.L.; and Pitts, R.F.: Interactions of Amino Acids During Renal Tubular Transport , Amer J Physiol 200:380-386 ( (Feb) ) 1961. 27. Webber, W.A.: Characteristics of Acidic Amino Acid Transport in Mammalian Kidney , Canad J Biochem Physiol 41:131-137 ( (Jan) ) 1963.Crossref 28. Scriver, C.R.; Pueschel, S.; and Davies, E.: Hyper-β-alaninemia Associated With β-aminoaciduria and y-Aminobutyricaciduria, Somnolence and Seizures , New Eng J Med 274:636-643 1966.Crossref 29. Gilbert, J.B., et al: The Increase in Urinary Taurine After Intraperitoneal Administration of Amino Acids to the Mouse , J Biol Chem 235:1055-1060 ( (April) ) 1960. 30. Goldman, H., and Scriver, C.: A Transport System in Mammalian Kidney With Preference for β-amino Compounds, abstracted , Ped Res 1: 212-213, 1967.Crossref 31. Knox, W.E.: " Cystinuria ," in Stanbury, J. B.; Wyngaarden, J.B.; and Fredrickson, D.S. (eds.): The Metabolic Basis of Inherited Disease , ed 2, New York: McGraw-Hill Book Co., Inc., 1966, pp 1262-1282. 32. Baron, D.N., et al: Hereditary Pellagra-like Skin Rash With Temporary Cerebellar Ataxia, Constant Renal Aminoaciduria and Other Bizarre Biochemical Features , Lancet 2:421-428 ( (Sept 1) ) 1956.Crossref 33. Jepson, J.B.: " Hartnup Disease ," in Stanbury, J.B.; Wyngaarden, J.B.; and Fredrickson, D.S. (eds.): The Metabolic Basis of Inherited Disease , ed 2, New York: McGraw-Hill Book Co., Inc., 1966, pp 1283-1299. 34. Milne, M.D., et al: The Metabolic Disorder in Hartnup Disease , Quart J Med 29:407-421 ( (July) ) 1960. 35. Scriver, C.R.: Hartnup Disease: A Genetic Modification of Intestinal and Renal Transport of Certain Neutral Alpha-Amino Acids , New Eng J Med 273:530-532 ( (Sept) ) 1965.Crossref 36. Scriver, C.R., and Wilson, O.H.: Amino Acid Transport in Human Kidney: Evidence for Genetic Control of Two Types , Science 155:1428-1430 ( (March) ) 1967.Crossref 37. Whelan, D.T., and Scriver, C.R.: Cystathioninuria and Renal Imino-Glycinuria in a Pedigree: A Perspective on Counselling , New Eng J Med 278: 924-927 ( (April) ) 1968.Crossref 38. Tada, K., et al: Prolinuria: A New Renal Tubular Defect in Transport of Proline and Glycine , J Exp Med 87:133-143 ( (Nov 25) ) 1965. 39. Goodman, S.I.; McIntyre, C.A.; and O'Brien, D.: Impaired Intestinal Transport of Proline in a Patient With Familial Iminoaciduria , J Pediat 71: 246-249 ( (Aug) ) 1967.Crossref 40. Rosenberg, L.E.; Devant, J.E.; and Elsas, L.J.: Familial Iminoglycinuria: An Inborn Error of Renal Tubular Transport , New Eng J Med 278: 1407-1413 ( (June) ) 1968.Crossref 41. Scriver, C.R.: Renal Tubular Transport of Proline, Hydroxyproline and Glycine: III. Genetic Basis for More Than One Mode of Uptake in Human Kidney , J Clin Invest 47:823-835 ( (April) ) 1968.Crossref 42. Doolan, P.D., et al: Renal Clearance of Lysine in Cystinuria , Amer J Med 23:416-425 ( (Sept) ) 1957.Crossref 43. Frimpter, G.E., et al: Inulin and Endogenous Amino Acid Renal Clearances in Cystinuria: Evidence for Tubular Secretion , J Clin Invest 41:281-288 ( (Feb) ) 1962.Crossref 44. Crawhall, J.C., et al: The Renal Clearance of Amino Acids in Cystinuria , J Clin Invest 43:1162-1171 ( (July) ) 1967.Crossref 45. Rosenberg, L.E.; Albrecht, I.; and Segal, S.: Lysine Transport in Human Kidney: Evidence for Two Systems , Science 155:1426-1428 ( (March) ) 1967.Crossref 46. Scriver, C.R., and Mohyuddin, F.: Amino Acid Transport in Kidney: Heterogeneity of AIB Uptake , J Biol Chem 243:3207-3213 ( (June) ) 1968. 47. Rosenberg, L.E.: Cystinuria: Genetic Heterogeneity and Allelism , Science 154:1341-1343 ( (Dec) ) 1966.Crossref 48. Segal, S., and Crawhall, J.C.: Characteristics of Cystine and Cysteine Transport in Rat Kidney Cortex Slices , Proc Nat Acad Sci 59:231-237 ( (Jan) ) 1968.Crossref 49. Schwartzman, L.; Blair, A,; and Segal, S.: Exchange Diffusion of Dibasic Amino Acids in Rat Kidney Cortex Slices , Biochim Biophys Acta 135: 120-136 ( (Feb 1) ) 1967.Crossref 50. Brodehl, J.; Gellisen, K.; and Kowalewski, S.: Isolierter Defekt der tubulären Cystin-Rück resorption in einer Familie mit idiopathischem Hypoparathyroidismus , Klin Wschr 45:38-40 ( (Jan 1) ) 1967.Crossref 51. Whelan, D.T., and Scriver, C.R.: Hyperdibasicaminoaciduria: An Inherited Disorder of Amino Acid Transport , Ped Res 2:523-532 ( (Nov) ) 1968.Crossref 52. Kekomäki, M., et al: Familial Protein Intolerance With Deficient Transport of Basic Amino Acids , Acta Paed Scand 56:617-630 ( (Nov) ) 1967.Crossref 53. Harris, H., et al: Pattern of Amino Acid Excretion in Cystinuria , Ann Hum Genet 19:195-208 ( (Feb) ) 1955.Crossref 54. Christensen, H.N.: Reactive Sites and Biological Transport , Advances Protein Chem 15:239-314, 1960. 55. Christensen, H.N.: Some Transport Lessons Taught by the Organic Solute , Perspect Biol Med 10:471-494 (No. (3) ) 1967. 56. Kepes, A.: " The Place of Permeases in Cellular Organization ," in J. F. Hoffman (ed.), The Cellular Functions of Membrane Transport , Englewood Cliffs, NJ: Prentice-Hall, Inc., 1964, pp 155-169. 57. Efron, M.L.: Treatment of Hydroxyprolinemia and Hyperprolinemia , Amer J Dis Child 113:166-169 ( (Jan) ) 1967. 58. Scriver, C.R.: Membrane Transport in Disorders of Imino Acid Metabolism , Amer J Dis Child 113:170-173 ( (Jan) ) 1967.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Jan 1, 1969

References