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Article |

Aminoaciduria-Renal Transport FREE

Charles E. Hollerman, MD; Philip L. Calcagno, MD
[+] Author Affiliations

Received for publication Oct 19, 1967.

Submitted by the authors for the Mitchell I. Rubin Festschrift issue of the JOURNAL.

Reprint requests to 3800 Reservoir Rd, Washington, DC 20007 (Dr. Hollerman).


Am J Dis Child. 1968;115(2):169-178. doi:10.1001/archpedi.1968.02100010171004.
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AMINO ACIDS in free form in the plasma are filtered through the glomerulus with almost complete tubular reabsorption (Fig 1). Aminoaciduria is a common finding in the majority of normal individuals accounting for 1% to 2% of the total urinary nitrogen excretion. The term aminoaciduria is commonly interpreted as the urinary excretion of one or more amino acids in quantities greater than normal or the excretion of certain amino acids or intermediates of amino acid metabolism not normally found in the urine or both.

Various classifications of aminoaciduria exist which involve terminology such as physiologic, primary or secondary, congenital or acquired, prerenal and renal, and combinations of the preceding. One classification based on the presumed or proven pathogenesis is as follows.

  1. Overflow or Prerenal Aminoaciduria. The increased urinary excretion of amino acids is regarded as a consequence of elevated plasma amino acid levels. The increased plasma values may be

REFERENCES

Efron, M.L.:  Aminoaciduria , New Eng J Med 272:1058-1067, 1965;.
Efron, M.L.:  Aminoaciduria , New Eng J Med 272:1107-1113, 1965;.
Nyhan, W.L., and Tocci, P.:  Aminoaciduria , Ann Rev Med 17:133-160, 1966;.
Berry, H.K.:  Individual Metabolic Patterns , Metabolism 9:363-372, 1960;.
Berry, H.K.:  Amino Acid Excretion in Urine of Normal Infants and Children , Pediatrics 25:983-996, 1960;.
Woolf, L.I., and Norman, A.P.:  The Urinary Excretion of Amino Acids and Sugars in Early Infancy , J Pediat 50:271-295, 1957;.
Childs, B.:  Urinary Excretion of Free Alpha-Aminonitrogen by Normal Infants and Children , Proc Soc Exp Biol Med 81:225-226, 1952;.
Ghadimi, H., and Shwachman, H.:  Evaluation of Aminoaciduria in Infancy and Childhood , Amer J Dis Child 99:457-475, 1960;.
Bloxam, H.R., et al:  An Inborn Defect in the Metabolism of Tyrosine in Infants on a Normal Diet , Biochem J 77:320-326, 1960;.
Brown, J.L.; Samiy, A.H.; and Pitts, R.F.:  Localization of Aminonitrogen Reabsorption in the Nephron of the Dog , Amer J Physiol 200:370-372, 1961;.
Ruszkowski, M., et al:  Renal Reabsorption of Amino Acids , Amer J Physiol 203:891-896, 1962;.
Pitts, R.F.:  A Renal Reabsorptive Mechanism in the Dog Common to Glycin and Creatine , Amer J Physiol 140:156-167, 1943;-1944.
Pitts, R.F.:  A Comparison of the Renal Reabsorptive Processes for Several Amino Acids , Amer J Physiol 140:535-547, 1943;-1944.
Wright, L.D., et al:  The Renal Clearance of Essential Amino Acids, Arginine, Histidine, Lysine and Methionine , Amer J Physiol 149:130-134, 1947;.
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;.
Coulson, R.A., and Hernandez, R.A.:  Amino Acid Metabolism in the Alligator , Fred Proc 24: 927-940, 1965;.
Udenfriend, S.; Zaltzman-Nirenberg, P.; and Guroff, G.:  A Study of Cellular Transport With the Fluorescent Amino Acid, Aminonaphthylalanine , Arch Biochem Biophy 116:261-270, 1966;.
Fox, M., et al:  Ionic Requirements for Amino Acid Transport in the Rat Kidney Cortex Slice , Biochim Biophys Acta 79:167-176, 1964;.
Finerman, G.A.M., and Rosenberg, L.E.:  Amino Acid Transport in Bone , J Biol Chem 241: 1487-1493, 1966;.
Bronk, J.R., and Parsons, D.S.:  Amino Acid Accumulation and Incorporation in Rat Intestine in Vitro , J Physiol 184:950-963, 1966;.
Wheeler, K.P., et al:  Relation of Amino Acid Transport to Sodium Ion Concentration , Biochim Biophys Acta 109:620-622, 1965;.
Schultz, S.G., and Zalusky, R.:  Ion Transport in Isolated Rabbit Ileum , J Gen Physiol 47:1043-1059, 1964;.
Schultz, S.G., and Zalusky, R.:  Interactions Between Active Sodium Transport and Active Amino Acid Transport in Isolated Rabbit Ileum , Nature 205:292-294, 1965;.
Jacobs, F.A.; Coen, L.J.; and Hillman, R.S.L.:  Influence of Pyridoxine, Pyridoxal Phosphate, Deoxypyridoxine and 2,4 Dinitrophenol on Methionine Absorption , J Biol Chem 235:1372-1375, 1960;.
Bittner, J., and Heinz, E.:  On the Effect of Pyridoxal on the Transport of Amino Acids in Ehrlich Mouse Ascites Tumor Cells (EMAT) , Biochem Biophys Res Commun 17:636-641, 1964;.
Abadom, P.N., and Scholefield, P.G.:  Amino Acid Transport in Brain Cortex Slices , Canad J Biochem 40:1575-1590, 1962;.
Abadom, P.N., and Scholefield, P.G.:  Amino Acid Transport in Brain Cortex Slices , Canad J Biochem 40:1603-1618, 1962;.
Jacquez, J.A.:  Carrier-Amino Acid Stoichiometry in Amino Acid Transport in Ehrlich Ascites Cells , Biochim Biophys Acta 71:15-33, 1963;.
Holden, J. (ed.): Amino Acid Pool , Elsevier Pub. Co., NY: 1962;, pp 815.
Scriver, C.R.:  "Hereditary Aminoaciduria,"  in Steinberg, A.G., and Bearn, A.G. (eds.): Progress in Medical Genetics , New York: Grune & Stratton, 1962;, vol 2, pp 83-186.
Christensen, H.N.:  Transport of Amino Acids , Nutr Rev 21:97-99, 1963;.
 Nutrition Symposium , Fed Proc 24:929-940, 1965;.
Segal, S.:  Disorders of Amino Acid Transport , Ann Intern Med 62:847-851, 1965;.
Milne, M.D.:  Disorders of Amino Acid Transport , Brit Med J 32:327-336, 1964;.
Scriver, C.R.; Pueschel, C.M.; and Davies, E.:  Hyper-β-Alaninemia Associated With β-Aminoaciduria and γ-Aminobutyricaciduria, Somnolence and Seizures , New Eng J Med 274:635-643, 1966;.
Wilson, T.H., et al:  Intestinal Transport of Sugars and Amino Acids , Fed Proc 19:870-875, 1960;.
Lin, E.C.C.; Hagihira, H.; and Wilson, T.H.:  Specificity of the Transport System for Neutral Amino Acids in the Hamster Intestine , Amer J Physiol 202:919-925, 1962;.
Randall, H.G., and Evered, D.F.:  Amino Acid Uptake by Living Cells , Biochim Biophys Acta 93: 98-105, 1964;.
Hagihira, H.; Wilson, T.H.; and Lin, E.C.C.:  Intestinal Transport of Certain N-Substituted Amino Acids , Amer J Physiol 203:637-640, 1962;.
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, 1956;.
Jepson, J.B.:  "Hartnup Disease,"  in Stanbury, J.B.; Wyngaarden, J.B.; and Frederickson, D.S. (eds.): The Metabolic Basis of Inherited Disease , ed 2, New York: McGraw-Hill Book Co., Inc., 1966;, pp 1283-1299.
Scriver, C.R.:  Hartnup Disease , New Eng J Med 273:530-532, 1965;.
McCarthy, C.F., et al:  Defective Uptake of Basic Amino Acids and L-Cystine by Intestinal Mucosa of Patients with Cystinuria , J Clin Invest 43:1518-1524, 1964;.
Thier, S., et al:  Cystinuria , Science 143:482-484, 1964;.
McLeod, M.E., and Tyor, M.P.:  Transport of Basic Amino Acids by Hamster Intestine , Amer J Physiol 213:163-168, 1967;.
Schwartzman, L.; Blair, A.; and Segal, S.:  A Common Renal Transport System for Lysine, Ornithine, Arginine, and Cystine , Biochem Biophys Res Commun 23:220-226, 1966;.
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;.
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, 1962;.
Fox, M., et al:  Evidence Against Single Transport Defect in Cystinuria , New Eng J Med 270: 556-561, 1964;.
Knox, W.E.: " Cystinuria ," in Stanbury, J.B.; Wyngaarden, J.B.; and Frederickson, D.S. (eds.): The Metabolic Basis of Inherited Disease , ed 2, New York: McGraw-Hill Book Co., Inc., 1966;, pp 1262-1282.
Stein, W.H.:  Excretion of Amino Acids in Cystinuria , Proc Soc Exp Biol Med 78:705-708, 1951;.
Yeh, H.L., et al:  The Urinary Excretion of Amino Acids by a Cystinuric Subject , Amer J Med Sci 214:507-512, 1947;.
Rosenberg, L.E., et al:  Cystinuria , J Clin Invest 45:365-371, 1966;.
Fleming, W.H., et al:  Gastrointestinal Malabsorption Associated With Cystinuria: Report of a Case in a Negro , Pediatrics 32:358-370, 1963;.
Perheentupa, J., and Visakorpi, J.K.:  Protein Intolerance With Deficient Transport of Basic Amino Acids , Lancet 2:813-816, 1965;.
Webber, W.A.:  Characteristics of Acidic Amino Acid Transport in Mammalian Kidney , Canad J Biochem Physiol 41:131-137, 1963;.
Wiseman, G.J.:  Preferential Transmission of Amino Acids From Amino Acid Mixtures by Sacs of Everted Small Intestine of the Golden Hamster , J Physiol 127:414-422, 1955;.
Wiseman, G.J.:  Active Transport of Amino Acids by Sacs of Everted Small Intestine of the Golden Hamster (Mesocricetus auratus) , J Physiol 133:626-630, 1956;.
Finch, L.R., and Hird, F.J.R.:  The Uptake of Amino Acids by Isolated Segment of Rat Intestine , Biochim Biophys Acta 43:278-287, 1960;.
Nathans, D.; Tapley, D.F.; and Ross, J.E.:  Intestinal Transport of Amino Acids Studied in Vitro With L- (131I) Monoiodotyrosine , Biochim Biophys Acta 41:271-282, 1960;.
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, 1961;.
Scriver, C.R.; Schafer, I.A.; and Efron, M.L.:  Evidence for a Renal Tubular Amino Acid Transport System Common to Glycine, L-Proline, and Hydroxy-L-Proline , abstracted, J Clin Invest 40: 1080-1081, 1961;.
Joseph, R., et al:  Maladie familial associant des convulsions á début trés précoce, une hyperaluminarchie et une hyperaminoacidurie , Arch Franc Pediat 15:374-387, 1958;.
Efron, M.L., et al:  Hydrodxyprolinemia Associated With Mental Deficiency , New Eng J Med 267:1193-1194, 1962;.
Efron, M.L.; Bixby, E.M.; and Pryles, C.V.:  Hydroxyprolinemia , New Eng J Med 272:1299-1309, 1965;.
De Vries, A., et al:  Glycinuria, Hereditary Disorder Associated With Nephrolithiasis , Amer J Med 23:408-415, 1957;.
Scriver, C.R., and Onslow, H.W.:  Possible Locations for a Common Gene Product in Membrane Transport of Imino Acids and Glycine , Nature 202:92-93, 1964;.
Scriver, C.R.:  Membrane Transport in Disorders of Imino Acid Metabolism , Amer J Dis Child 113:170-174, 1967;.
Dent, C.E.:  Amino-Aciduria in Fanconi Syndrome , Biochem J 41:240-253, 1947;.
Clarkson, T.W., and Kench, J.E.:  Urinary Excretion of Amino Acids by Men Absorbing Heavy Metals , Biochem J 62:361-372, 1956;.
Chisolm, J.J., Jr.:  Aminoaciduria as a Manifestation of Renal Tubular Injury in Lead Intoxication and a Comparison With Patterns of Aminoaciduria Seen in Other Disease , J Pediat 60: 1-17, 1962;.
Harrison, H.E., and Harrison, H.C:  Experimental Production of Renal Glycosuria, Phosphaturia, and Aminoaciduria by Injection of Maleic Acid , Science 120:606-608, 1954;.
Rosenberg, L.E., and Segal, S.:  Maleic Acid-Induced Inhibition of Amino Acid Transport in Rat Kidney , Biochem J 92:345-352, 1964;.
Frimpter, G.W., et al:  Reversible "Fanconi Syndrome" Caused by Degraded Tetracycline , JAMA 184:111-113, 1963;.
Cusworth, D.C; Dent, C.E.; and Flynn, F.V.:  Amino-Aciduria in Galactosemia , Arch Dis Child 30:150-154, 1955;.
Bearn, A.G.:  Wilson's Disease , Amer J Med 22:747-757, 1957;.
Richards, W., et al:  Oculo-Cerebro-Renal Syndrome of Lowe , Amer J Dis Child 100:707-709, 1960;.
Jonxis, J.H.P., and Huisman, T.H.J.:  Aminoaciduria and Ascorbic Acid Deficiency , Pediatrics 14:238-244, 1954;.
Jonxis, J.H.P., and Huisman, T.H.J.:  Rickets and Amino-Aciduria , Lancet 2:1015-1017, 1952;.
Worthen, H.G.:  Effect of Maleic Acid on Renal Tubular Enzyme Activity and Morphology , abstracted, Fed Proc 20:408, 1960;.
Thier, S., et al:  Hexose Inhibition of Amino Acid Uptake on Rat-Kidney Cortex Slice , Biochim Biophys Acta 93:106-115, 1964;.
Käser, H.; Cottier, P.; and Antener, I.:  Glucoglycinuria , J Pediat 61:386-394, 1962;.
Brown, D.M., and Michael, A.F.:  Inhibition of Inorganic Phosphate Transport by Amino Acids in Rat Kidney Cortical Slices in Vitro , Proc Soc Exp Biol Med 124:470-475, 1967;.
Dent, C.E., and Harris, H:  Hereditary Forms of Rickets and Osteomalacia , J Bone Joint Surg 38B:204-226, 1956;.
Bessman, S.P., and Baldwin, R.:  Imidazole Aminoaciduria in Cerebromacular Degeneration , Science 135:789-791, 1962;.
Efron, M.L., et al:  A Simple Chromatographic Screening Test for the Detection of Disorders of Amino Acid Metabolism , New Eng J Med 270:1378- 1383, 1964;.

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References

Efron, M.L.:  Aminoaciduria , New Eng J Med 272:1058-1067, 1965;.
Efron, M.L.:  Aminoaciduria , New Eng J Med 272:1107-1113, 1965;.
Nyhan, W.L., and Tocci, P.:  Aminoaciduria , Ann Rev Med 17:133-160, 1966;.
Berry, H.K.:  Individual Metabolic Patterns , Metabolism 9:363-372, 1960;.
Berry, H.K.:  Amino Acid Excretion in Urine of Normal Infants and Children , Pediatrics 25:983-996, 1960;.
Woolf, L.I., and Norman, A.P.:  The Urinary Excretion of Amino Acids and Sugars in Early Infancy , J Pediat 50:271-295, 1957;.
Childs, B.:  Urinary Excretion of Free Alpha-Aminonitrogen by Normal Infants and Children , Proc Soc Exp Biol Med 81:225-226, 1952;.
Ghadimi, H., and Shwachman, H.:  Evaluation of Aminoaciduria in Infancy and Childhood , Amer J Dis Child 99:457-475, 1960;.
Bloxam, H.R., et al:  An Inborn Defect in the Metabolism of Tyrosine in Infants on a Normal Diet , Biochem J 77:320-326, 1960;.
Brown, J.L.; Samiy, A.H.; and Pitts, R.F.:  Localization of Aminonitrogen Reabsorption in the Nephron of the Dog , Amer J Physiol 200:370-372, 1961;.
Ruszkowski, M., et al:  Renal Reabsorption of Amino Acids , Amer J Physiol 203:891-896, 1962;.
Pitts, R.F.:  A Renal Reabsorptive Mechanism in the Dog Common to Glycin and Creatine , Amer J Physiol 140:156-167, 1943;-1944.
Pitts, R.F.:  A Comparison of the Renal Reabsorptive Processes for Several Amino Acids , Amer J Physiol 140:535-547, 1943;-1944.
Wright, L.D., et al:  The Renal Clearance of Essential Amino Acids, Arginine, Histidine, Lysine and Methionine , Amer J Physiol 149:130-134, 1947;.
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;.
Coulson, R.A., and Hernandez, R.A.:  Amino Acid Metabolism in the Alligator , Fred Proc 24: 927-940, 1965;.
Udenfriend, S.; Zaltzman-Nirenberg, P.; and Guroff, G.:  A Study of Cellular Transport With the Fluorescent Amino Acid, Aminonaphthylalanine , Arch Biochem Biophy 116:261-270, 1966;.
Fox, M., et al:  Ionic Requirements for Amino Acid Transport in the Rat Kidney Cortex Slice , Biochim Biophys Acta 79:167-176, 1964;.
Finerman, G.A.M., and Rosenberg, L.E.:  Amino Acid Transport in Bone , J Biol Chem 241: 1487-1493, 1966;.
Bronk, J.R., and Parsons, D.S.:  Amino Acid Accumulation and Incorporation in Rat Intestine in Vitro , J Physiol 184:950-963, 1966;.
Wheeler, K.P., et al:  Relation of Amino Acid Transport to Sodium Ion Concentration , Biochim Biophys Acta 109:620-622, 1965;.
Schultz, S.G., and Zalusky, R.:  Ion Transport in Isolated Rabbit Ileum , J Gen Physiol 47:1043-1059, 1964;.
Schultz, S.G., and Zalusky, R.:  Interactions Between Active Sodium Transport and Active Amino Acid Transport in Isolated Rabbit Ileum , Nature 205:292-294, 1965;.
Jacobs, F.A.; Coen, L.J.; and Hillman, R.S.L.:  Influence of Pyridoxine, Pyridoxal Phosphate, Deoxypyridoxine and 2,4 Dinitrophenol on Methionine Absorption , J Biol Chem 235:1372-1375, 1960;.
Bittner, J., and Heinz, E.:  On the Effect of Pyridoxal on the Transport of Amino Acids in Ehrlich Mouse Ascites Tumor Cells (EMAT) , Biochem Biophys Res Commun 17:636-641, 1964;.
Abadom, P.N., and Scholefield, P.G.:  Amino Acid Transport in Brain Cortex Slices , Canad J Biochem 40:1575-1590, 1962;.
Abadom, P.N., and Scholefield, P.G.:  Amino Acid Transport in Brain Cortex Slices , Canad J Biochem 40:1603-1618, 1962;.
Jacquez, J.A.:  Carrier-Amino Acid Stoichiometry in Amino Acid Transport in Ehrlich Ascites Cells , Biochim Biophys Acta 71:15-33, 1963;.
Holden, J. (ed.): Amino Acid Pool , Elsevier Pub. Co., NY: 1962;, pp 815.
Scriver, C.R.:  "Hereditary Aminoaciduria,"  in Steinberg, A.G., and Bearn, A.G. (eds.): Progress in Medical Genetics , New York: Grune & Stratton, 1962;, vol 2, pp 83-186.
Christensen, H.N.:  Transport of Amino Acids , Nutr Rev 21:97-99, 1963;.
 Nutrition Symposium , Fed Proc 24:929-940, 1965;.
Segal, S.:  Disorders of Amino Acid Transport , Ann Intern Med 62:847-851, 1965;.
Milne, M.D.:  Disorders of Amino Acid Transport , Brit Med J 32:327-336, 1964;.
Scriver, C.R.; Pueschel, C.M.; and Davies, E.:  Hyper-β-Alaninemia Associated With β-Aminoaciduria and γ-Aminobutyricaciduria, Somnolence and Seizures , New Eng J Med 274:635-643, 1966;.
Wilson, T.H., et al:  Intestinal Transport of Sugars and Amino Acids , Fed Proc 19:870-875, 1960;.
Lin, E.C.C.; Hagihira, H.; and Wilson, T.H.:  Specificity of the Transport System for Neutral Amino Acids in the Hamster Intestine , Amer J Physiol 202:919-925, 1962;.
Randall, H.G., and Evered, D.F.:  Amino Acid Uptake by Living Cells , Biochim Biophys Acta 93: 98-105, 1964;.
Hagihira, H.; Wilson, T.H.; and Lin, E.C.C.:  Intestinal Transport of Certain N-Substituted Amino Acids , Amer J Physiol 203:637-640, 1962;.
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, 1956;.
Jepson, J.B.:  "Hartnup Disease,"  in Stanbury, J.B.; Wyngaarden, J.B.; and Frederickson, D.S. (eds.): The Metabolic Basis of Inherited Disease , ed 2, New York: McGraw-Hill Book Co., Inc., 1966;, pp 1283-1299.
Scriver, C.R.:  Hartnup Disease , New Eng J Med 273:530-532, 1965;.
McCarthy, C.F., et al:  Defective Uptake of Basic Amino Acids and L-Cystine by Intestinal Mucosa of Patients with Cystinuria , J Clin Invest 43:1518-1524, 1964;.
Thier, S., et al:  Cystinuria , Science 143:482-484, 1964;.
McLeod, M.E., and Tyor, M.P.:  Transport of Basic Amino Acids by Hamster Intestine , Amer J Physiol 213:163-168, 1967;.
Schwartzman, L.; Blair, A.; and Segal, S.:  A Common Renal Transport System for Lysine, Ornithine, Arginine, and Cystine , Biochem Biophys Res Commun 23:220-226, 1966;.
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;.
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, 1962;.
Fox, M., et al:  Evidence Against Single Transport Defect in Cystinuria , New Eng J Med 270: 556-561, 1964;.
Knox, W.E.: " Cystinuria ," in Stanbury, J.B.; Wyngaarden, J.B.; and Frederickson, D.S. (eds.): The Metabolic Basis of Inherited Disease , ed 2, New York: McGraw-Hill Book Co., Inc., 1966;, pp 1262-1282.
Stein, W.H.:  Excretion of Amino Acids in Cystinuria , Proc Soc Exp Biol Med 78:705-708, 1951;.
Yeh, H.L., et al:  The Urinary Excretion of Amino Acids by a Cystinuric Subject , Amer J Med Sci 214:507-512, 1947;.
Rosenberg, L.E., et al:  Cystinuria , J Clin Invest 45:365-371, 1966;.
Fleming, W.H., et al:  Gastrointestinal Malabsorption Associated With Cystinuria: Report of a Case in a Negro , Pediatrics 32:358-370, 1963;.
Perheentupa, J., and Visakorpi, J.K.:  Protein Intolerance With Deficient Transport of Basic Amino Acids , Lancet 2:813-816, 1965;.
Webber, W.A.:  Characteristics of Acidic Amino Acid Transport in Mammalian Kidney , Canad J Biochem Physiol 41:131-137, 1963;.
Wiseman, G.J.:  Preferential Transmission of Amino Acids From Amino Acid Mixtures by Sacs of Everted Small Intestine of the Golden Hamster , J Physiol 127:414-422, 1955;.
Wiseman, G.J.:  Active Transport of Amino Acids by Sacs of Everted Small Intestine of the Golden Hamster (Mesocricetus auratus) , J Physiol 133:626-630, 1956;.
Finch, L.R., and Hird, F.J.R.:  The Uptake of Amino Acids by Isolated Segment of Rat Intestine , Biochim Biophys Acta 43:278-287, 1960;.
Nathans, D.; Tapley, D.F.; and Ross, J.E.:  Intestinal Transport of Amino Acids Studied in Vitro With L- (131I) Monoiodotyrosine , Biochim Biophys Acta 41:271-282, 1960;.
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, 1961;.
Scriver, C.R.; Schafer, I.A.; and Efron, M.L.:  Evidence for a Renal Tubular Amino Acid Transport System Common to Glycine, L-Proline, and Hydroxy-L-Proline , abstracted, J Clin Invest 40: 1080-1081, 1961;.
Joseph, R., et al:  Maladie familial associant des convulsions á début trés précoce, une hyperaluminarchie et une hyperaminoacidurie , Arch Franc Pediat 15:374-387, 1958;.
Efron, M.L., et al:  Hydrodxyprolinemia Associated With Mental Deficiency , New Eng J Med 267:1193-1194, 1962;.
Efron, M.L.; Bixby, E.M.; and Pryles, C.V.:  Hydroxyprolinemia , New Eng J Med 272:1299-1309, 1965;.
De Vries, A., et al:  Glycinuria, Hereditary Disorder Associated With Nephrolithiasis , Amer J Med 23:408-415, 1957;.
Scriver, C.R., and Onslow, H.W.:  Possible Locations for a Common Gene Product in Membrane Transport of Imino Acids and Glycine , Nature 202:92-93, 1964;.
Scriver, C.R.:  Membrane Transport in Disorders of Imino Acid Metabolism , Amer J Dis Child 113:170-174, 1967;.
Dent, C.E.:  Amino-Aciduria in Fanconi Syndrome , Biochem J 41:240-253, 1947;.
Clarkson, T.W., and Kench, J.E.:  Urinary Excretion of Amino Acids by Men Absorbing Heavy Metals , Biochem J 62:361-372, 1956;.
Chisolm, J.J., Jr.:  Aminoaciduria as a Manifestation of Renal Tubular Injury in Lead Intoxication and a Comparison With Patterns of Aminoaciduria Seen in Other Disease , J Pediat 60: 1-17, 1962;.
Harrison, H.E., and Harrison, H.C:  Experimental Production of Renal Glycosuria, Phosphaturia, and Aminoaciduria by Injection of Maleic Acid , Science 120:606-608, 1954;.
Rosenberg, L.E., and Segal, S.:  Maleic Acid-Induced Inhibition of Amino Acid Transport in Rat Kidney , Biochem J 92:345-352, 1964;.
Frimpter, G.W., et al:  Reversible "Fanconi Syndrome" Caused by Degraded Tetracycline , JAMA 184:111-113, 1963;.
Cusworth, D.C; Dent, C.E.; and Flynn, F.V.:  Amino-Aciduria in Galactosemia , Arch Dis Child 30:150-154, 1955;.
Bearn, A.G.:  Wilson's Disease , Amer J Med 22:747-757, 1957;.
Richards, W., et al:  Oculo-Cerebro-Renal Syndrome of Lowe , Amer J Dis Child 100:707-709, 1960;.
Jonxis, J.H.P., and Huisman, T.H.J.:  Aminoaciduria and Ascorbic Acid Deficiency , Pediatrics 14:238-244, 1954;.
Jonxis, J.H.P., and Huisman, T.H.J.:  Rickets and Amino-Aciduria , Lancet 2:1015-1017, 1952;.
Worthen, H.G.:  Effect of Maleic Acid on Renal Tubular Enzyme Activity and Morphology , abstracted, Fed Proc 20:408, 1960;.
Thier, S., et al:  Hexose Inhibition of Amino Acid Uptake on Rat-Kidney Cortex Slice , Biochim Biophys Acta 93:106-115, 1964;.
Käser, H.; Cottier, P.; and Antener, I.:  Glucoglycinuria , J Pediat 61:386-394, 1962;.
Brown, D.M., and Michael, A.F.:  Inhibition of Inorganic Phosphate Transport by Amino Acids in Rat Kidney Cortical Slices in Vitro , Proc Soc Exp Biol Med 124:470-475, 1967;.
Dent, C.E., and Harris, H:  Hereditary Forms of Rickets and Osteomalacia , J Bone Joint Surg 38B:204-226, 1956;.
Bessman, S.P., and Baldwin, R.:  Imidazole Aminoaciduria in Cerebromacular Degeneration , Science 135:789-791, 1962;.
Efron, M.L., et al:  A Simple Chromatographic Screening Test for the Detection of Disorders of Amino Acid Metabolism , New Eng J Med 270:1378- 1383, 1964;.

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