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Amino Acids in Health and Disease

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Nutritional Elements and Clinical Biochemistry

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Abstract

The proteins of the human body are in a constant state of flux with some proteins being synthesized and catabolized continually. Dietary proteins and tissue protein breakdown products provide a pool of amino acids which provide the building blocks for formation of new protein for growth and for rebuilding tissue protein. Essential amino acids are those not synthesized by man or not produced sufficiently rapidly enough to support this activity. Therefore, man requires a source of nitrogen and essential amino acids to support growth, protein synthesis and biosynthesis of important nitrogenous metabolites. The differences in value of food proteins are due to variations in amino acid pattern of each protein. Since the body can form only some of the amino acids, nine “essential” ones must be supplied in the diet (Table I) (McLaughlan et al., 1963, Schelling, 1975). Protein normally is not absorbed by the human intestine, but its hydrolysis products peptides and free amino acids are readily absorbed by the small intestine and transported via the portal blood. A diet low in protein or food protein which is deficient in one or more essential amino acids will restrict protein synthesis. Nonessential amino acids are generally metabolized to pyruvate which leads to gluconeogensis. Therefore, in times of great need (starvation) body proteins can provide energy via glucose. The problem here is that the conversion of protein to glucose is only about 50% efficient (Bessman, 1975) so that 100 g of glucose will require 200 g of protein or the equivalent of 1 kg of lean body weight. Most of the essential amino acids are converted to acetyl CoA and their nitrogen as well as that of the amino acids converted to glucose is lost to the body primarily as ammonia.

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References

  • Abitol, C. L., Feldman, D. B., Ahmann, P. and Rudman, D., 1975, Plasma amino acid patterns during supplemental intravenous nutrition of low-birth weight infants, Pediat., 86:766.

    Article  Google Scholar 

  • Adibi, S. A., Drash, A. L., and Livi, E. D., 1970, Hormone and amino acid levels in altered nutritional states, J. Lab. Clin. Med., 76:722.

    Google Scholar 

  • Aoki, T. T., Manzano, M., Kozak, G. P., Cahill, G. F., Jr., 1975, Plasma and cerebrospinal fluid amino acid levels in diabetic ketoacidosis before and after corrective therapy, J. Amer. Diabetes Ass., 24:463.

    Article  Google Scholar 

  • Armstrong, M. D. and Stave, U., 1973, A study of free amino acid levels I. Study of factors affecting the validity of amino acid analysis, Metab., 22:549.

    Article  Google Scholar 

  • Arroyove, G., Wilson, D., DeFunes, C. and Behar, M., 1962, The free amino acids in blood plasma of children with kwashiorkor and marasmus, Am. J. Clin. Nutr., 11:517.

    Google Scholar 

  • Arroyove, G. and Bowering, J., 1968, Plasma free amino acids as an index of protein nutrition, Arch. Latin. deNutr., 23:341.

    Google Scholar 

  • Baertle, J. M., Placko, R. P. and Graham, G. G., 1974, Serum proteins and free amino acids in severe malnutrition, Am. J. Clin. Nutr., 27:733.

    Google Scholar 

  • Bessman, S. P., 1975, Interrelationships of various food materials in total parenteral nutrition, (H. Ghadimi, ed.) John Wiley and Sons, Inc.

    Google Scholar 

  • Bessman, S. P., 1972, Genetic failure of fetal amino acid justification: A common basis for many forms of metabolic, nutritional and “nonspecific” mental retardation, J. Pediat., 81:834.

    Article  Google Scholar 

  • Brans, Y. W., Sumners, J. E., Dweck, H. S. and Cassady, G., 1974, Feeding the low birth weight infant: Orally or parenterally. Preliminary results of a comparative study, Pediat., 54:15.

    Google Scholar 

  • Cahill, G. F., Jr., 1970, Starvation in man, N. Eng. J. Med., 282:668.

    Article  Google Scholar 

  • Dent, C. E., 1946, Detection of amino acid in urine and other fluids, Lancet, (ii):637.

    Google Scholar 

  • Dickinson, J. S., Rosenblum, H., Hamilton, P. B., 1965, Ion exhange chromatography of the free amino acids in the plasma of the newborn infant, Pediat., 36:2.

    Google Scholar 

  • Dirren, H., Robinson, A. B., Pauling, L., 1975, Sex related patterns in the profiles of human urinary amino acids, Clin. Chem., 21, 1970.

    Google Scholar 

  • Efron, M. L., 1959, Two-way separation of amino acids and other ninhydrin reacting substances by high voltage electrophoresis followed by paper chromatography, Biochem. J., 72:691.

    Google Scholar 

  • Efron, M. L., Young, D., Moser, H. W. and McCready, R. A., 1964, A simple chromatographic screening test for the detection of disorders of amino acid metabolism, N. Eng. J. Med., 270:1378.

    Article  Google Scholar 

  • Efron, M. L., 1968, High voltage paper electrophoresis in (I. Smith, ed.) Chromatographic and Electrophoretic Techniques, Vol. II, Second Edition (pp. 166–193) Interscience, New York.

    Google Scholar 

  • Feigin, R. D., Klainer, A. S., Beisel, W. R., 1968, Factors affecting ciradian periodicity of blood amino acids in man, Metab. J.

    Google Scholar 

  • Feigin, R. D. and Haymond, M. W., 1970, Circadian periodicity of blood amino acids in the neonate, Pediat., 45:782.

    Google Scholar 

  • Felig, P., Owen, P. E., Warrem, J. and Cahill, G. F., Jr., 1969, Amino acid metabolism during prolonged starvation, J. Clin. Invest., 48:584.

    Article  Google Scholar 

  • Felig, P., Marliss, E. and Cahill, G. F., Jr., 1969, Plasma amino acid levels and insulin secretion in obesity, N. Eng. J. Med., 281:811.

    Article  Google Scholar 

  • Felig, P., Marliss, E., Ohman, L. and Cahill, G. F., Jr., 1970, Plasma acid levels in diabetic ketoacidosis, Diabetes, 19:412.

    Google Scholar 

  • Felts, J. H., King, J. J., Jr., 1971, Enhanced secretion of free amino acids by hyperthyroidism patients, Clin. Chem., 17:388.

    Google Scholar 

  • Furst, P., Ahlberg, M., Alvestrand, A., Bergstrom, J., 1978, Principles of essential amino acid therapy in uremia. Am. J. Clin. Nutr., 31:1744.

    Google Scholar 

  • Garrod, A. E., 1908, The croonian lectures, Lancet, (ii).

    Google Scholar 

  • Gehrke, C. W., Roach, D., Zumwalt, R. W. and Stalling, D. L., 1968, Quantitative gas liquid chromatography of amino acids in proteins and biological substances, analytical biochemistry, Inc., Columbia.

    Google Scholar 

  • Gerristen, T., Rekberg, M. L., Waisman, H., 1965, On the determination of free amino acid levels in serum, Anal. Biochem., 11:460.

    Article  Google Scholar 

  • Ghadimi, H., Abaci, F., Kuman, S. and Rathi, M., 1971, Biochemical aspects of intravenous alimentation, Pediatr., 48:955.

    Google Scholar 

  • Ghadimi, H., 1973, A review: Current status of parental amino acid therapy, Pediat. Res., 7:169.

    Google Scholar 

  • Guthrie, R. and Susi, A., 1963, A Simple method for determining phenylalanine in large populations of newborn infants, Pediat., 32:338.

    Google Scholar 

  • Heard, C. R. C., Kriegsman, S. M. and Pratt, B. S., 1969, The interpretation of plasma amino acid ratios in protein calorie deficiency, Br. J. Nutr., 23:203.

    Article  Google Scholar 

  • Heird, W. C. and Winters, R. W., 1975, Total Parenteral Nutrition, J. Pediat., 86:2.

    Article  Google Scholar 

  • Hill, J. B., Summer, G. K., Pender, M. W. and Roszel, N. O., 1965, An automated procedure for blood phenylalanine, Clin. Chem., 11:541.

    Google Scholar 

  • Hilty, M. D., Romshe, C. A. and Delameter, P. V., 1974, Reyes snydrome and hyperaminoacidemia, J. Pediat., 84:362.

    Article  Google Scholar 

  • Hochella, N. J., 1967, Automated fluorometric determination in blood, Anal. Biochem., 21:227.

    Article  Google Scholar 

  • Holt, L. E., Jr., Gregory, P., Pratt, E. L., Snyderman, S. E., Wallace, W. M., 1960, Protein and amino acid requirements in early life, New York University Press, New York.

    Google Scholar 

  • Holt, L. E., Snyderman, Norton, P. M., Roitman, E. and Finch, J., 1963, The plasma aminogram in kwashiorkor, Lancet, (ii) 1344.

    Google Scholar 

  • Horning, E. C. and Horning, M. G., 1971, Human metabolic profiles obtained by GC and GC/MS, J. Chrom. Sci., 9:129.

    Google Scholar 

  • Jackson, H., Sardharwalla, I. B. and Ebers, G. C., 1968, Two systems of amino acid chromatography suitable for mass screening, Clin. Biochem., 2:163.

    Article  Google Scholar 

  • Kisser, W., 1974, Amino acid determinations in the serum of patients with multiple sclerosis, Anal. Chem., 86:3.

    Google Scholar 

  • Levy, H. L., Shih, V. E., Madigan, P., Karolkewics and MacCready, R. A., 1968, Results of a screening method for amino acids. I. Whole Blood, Clin. Biochem., 1:200.

    Article  Google Scholar 

  • Levy, H. L. and Barkin, E., 1971, Comparison of amino acid concentration between plasma and erythrocytes, Lab. Clin. Med., 78:517.

    Google Scholar 

  • Linblad, B. S., 1970, The venous plasma free amino acid levels during the first several hours of life. I. After normal and short gestation complicated by hypertension with special reference to the small dates syndrome, Acta. Paediat. Scan., 59:13.

    Article  Google Scholar 

  • Linblad, B. S. and Baldesten, A., 1969, Time studies on free amino acid levels of venous plasma during the neonate period, Acta. Paediat. Scan., 58:252.

    Article  Google Scholar 

  • Logan, R. W., Young, D. G., Ross, D. A., Stewart, B. R., Kubo, M. and Tryfonas, G., 1974, Comparison of an oral and intravenous regimen in the newborn, Arch. Dis. or Childhood, 49:200.

    Article  Google Scholar 

  • McCaman, M. W., and Robins, E., 1962, Fluorometric method for the determinations of phenylalanine in serum, J. Lab. Clin. Med., 59:885.

    Google Scholar 

  • McGale, E. H. F., Pickard, J. C., Aber, G., 1972, Quantitative changes of amino acids in patients with renal disease, Clin. Chem., 38:395.

    Article  Google Scholar 

  • McLaughlan, J. M., Noel, F. J., Morrison, A. B. and Campbell, J. A., 1963, “Methodology of protein evaluation” in (Munroe, A. N. and Allison, J. B., eds.), Mammalian protein in metabolism, II. 391–423, Academic Press, New York, New York.

    Google Scholar 

  • Mestyan, J., Feketa, M., Jarari, I., Sulyak, Imkoff, S. and Soltzberg, G. Y., 1969, The post natal changes in the circulating free amino acid pool in the newborn infant. II. The plasma amino acid ratio in intra-uterine malnutrition, Biol. Neonat., 14:164.

    Article  Google Scholar 

  • Moghissi, K. S., Churchill, J. A., Kurrie, D., 1975, Relationships of maternal amino acids and proteins to fetal growth and mental development, Amer. J. Obstet. Gynecol., 123:398.

    Google Scholar 

  • Moore, S. and Stein, W. H., 1954, Chromatography of amino acids on sulfonated polystyrene resins, J. Biol. Chem., 211:893.

    Google Scholar 

  • Monaco, F., Mutani, R., Durrelli, L., Delsedime, M., 1975, Free amino acids of patients with epilepsy: Significant increases of taurine, Epilepsia, 16:245.

    Article  Google Scholar 

  • Naeye, R. L., Blan, W. L., Paul C., 1973, Effects of maternal nutrition on the human fetus, Pediat., 54:494.

    Google Scholar 

  • National Academy of Sciences, 1968, (National Research Council): Recommended Dietary Allowances. The Academy-The Council: Wash.

    Google Scholar 

  • Nyhan, W. L., Tocci, P. M., 1966, Aminoaciduria, Ann. Rev. Med., 17:133.

    Article  Google Scholar 

  • Owen, O. E., Morgan, A. P., Kemp, H. G., Sullivan, J. M., Herrera, M. G., Cahill, C. G., Snyderman, S. E. and Norton, P. M., 1967, Brain metabolism during fasting, J. Clin. Invest., 46:15:89.

    Google Scholar 

  • Perry, T. L., and Hansen, S., 1969, Technical pitfalls leading to errors in the quantitation of plasma amino acids, Clin. Chem. Acta, 25:43.

    Article  Google Scholar 

  • Raine, D. N., Cooke, J. R., Andrews, W. A. and Mahon, D. F., 1972, Screening for inherited metabolic diseases by plasma chromatography in a large city, Brit. Med. J., 3:7.

    Article  Google Scholar 

  • Reid, D. W. J., Campbell, D. J., and Yakymyshyn, L. Y., 1971, Quantitative amino acids in amniotic fluids and maternal plasma in early and late pregnancy, Am. J. Obstet. Gynec., 111:251.

    Google Scholar 

  • Rosenblatt, D. and Scriver, C. P., 1968, Heterogeneity in genetic control of phenylalanine metabolism in man, Nature (London), 218:677.

    Article  Google Scholar 

  • Rouser, G., Jeliner, B., Samuels, A. J., Kinugasa, K., Holden, J. T., 1962, “Free amino acids in the blood of man and animals” in Amino Acid Pools; (J. T. Holden, ed.) Elsevier, New York.

    Google Scholar 

  • Rutishauser, I. H. E. and Whitehead, R. G., 1969, Field evaluation of two biochemical tests which may reflect nutritional status in three areas of Uganda, Br. J. Nutr., 23:1.

    Article  Google Scholar 

  • Schelling, G. T., 1975, “An efficient procedure for complete evaluation of dietary proteins” in Nutrition and Clinical Nutrition, Vol. I, Protein Nutritional Quality of Foods and Feeds, Part I, (M. Friedman, ed.) Marcel Deker, Inc., New York.

    Google Scholar 

  • Scriver, C. R., 1969, The human biochemical genetics of amino acid transport, Pediatr., 44:348.

    Google Scholar 

  • Segal, S., 1976, Disorders of renal amino acid transport, N. Eng. J. Med., 294:1044.

    Article  Google Scholar 

  • Sherwood, L. M., Parris, E. E., 1969, Inherited aminoacidopathies demonstrating vitamin dependency, N. Eng. J. Med., 281:145.

    Article  Google Scholar 

  • Soupart, P., 1962, “Free amino acids of blood and urine in the human” in Amino Acid Pools, (J. T. Holden, ed.), Elsevier, New York.

    Google Scholar 

  • Turner, B., and Brown, D. A., 1970, Amino acid excretion during infancy and early childhood, Med. J. Aus., 1:11.

    Google Scholar 

  • Tweedle, D. E. F., Spivey, J., and Johnson, I. D. A., 1973, Choice of intravenous amino acid for use after surgical operations, Metab., 22:173.

    Article  Google Scholar 

  • Wehr, R. F., and Lewis, G. T., 1966, Amino acids in blood plasma of young and aged adults, Soc. for Exper. Bio. Med., 121:349.

    Google Scholar 

  • Weller, L. A., Margen, S., Calloway, D. H., 1969, Variation in fasting and postprandial amino acids of men fed adequate or protein free diets, Am. J. Clin. Nutr., 22:1557.

    Google Scholar 

  • Westall, R. G., Roitman, E., de la Pena, C., Rasmussen, H. R., Cravioto, J., Gomez, F., and Holt, L. E., 1958, The plasma amino acids in malnutrition, Arch. Dis. Child, 23:499.

    Article  Google Scholar 

  • Whitehead, R. G. and Dean, R. F. A., 1964, Serum amino acids in kwashiorkor. II. An abbreviated method of estimation and its application, Am. J. Nutr., 14:320.

    Google Scholar 

  • Whitehead, R. G., 1967, Biochemical tests in differential diagnosis of protein and calorie deficiencies, Arch. Dis. Child., 42:479.

    Article  Google Scholar 

  • Wurtman, R. J., Rose, C. M., Chou, Chaun, C., Larin, F. F., 1968, Daily rhythms in the concentration of various amino acids in human plasma, N. Eng. J. Med., 279:171.

    Article  Google Scholar 

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Authors and Affiliations

  1. Biochemical Genetics Laboratory, Mailman Child Development Center, Department of Pediatrics, University of Miami, Miami, Florida, USA

    Paul M. Tocci Ph.D. (Director)

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  1. Paul M. Tocci Ph.D.
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Editors and Affiliations

  1. Departments of Pathology, Biochemistry, and Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA

    Marge A. Brewster

  2. Metabolic Laboratory, Arkansas Children’s Hospital, Little Rock, Arkansas, USA

    Marge A. Brewster

  3. Department of Biochemistry, Division of Laboratory Medicine, Department of A therosclerosis and Thrombosis Research, Division of Research, Cleveland Clinic Foundation, Cleveland, Ohio, USA

    Herbert K. Naito

  4. Department of Chemistry, Cleveland State University, Cleveland, Ohio, USA

    Herbert K. Naito

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© 1980 Plenum Press, New York

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Tocci, P.M. (1980). Amino Acids in Health and Disease. In: Brewster, M.A., Naito, H.K. (eds) Nutritional Elements and Clinical Biochemistry. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-3168-1_14

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  • DOI: https://doi.org/10.1007/978-1-4613-3168-1_14

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-3170-4

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