Disorders of Amino Acid Transport
Authors: STANTON SEGAL
Journal: Annals of Internal Medicine (1965)

Editorials1 April 1965Disorders of Amino Acid TransportSTANTON SEGAL, M.D.STANTON SEGAL, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-62-4-847 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptWithin recent years authorities on cystinuria have concluded that renal clearance evidence has clarified the pathogenesis of this hereditary disease (1, 2). The National Institutes of Health (NIH) Clinical Staff Conference on cystinuria in this issue of the ANNALS is an excellent example of how the application of new investigative techniques and illuminated thought can make a seemingly well-understood disease the subject of fruitful research. The use of D-penicillamine to prevent cystine stone formation not only provides useful therapy but will, no doubt, further the insight into the underlying defect as a result of investigative efforts to understand some of...References1. KNOX WE: Cystinuria, in The Metabolic Basis of Inherited Disease. Edited by STANBURY, J., WYNGAARDEN, J. B., FREDERICKSON, D. S., McGraw-Hill, New York, 1960, pp. 1302-1337. Google Scholar2. HARRIS H: Inborn errors today, in Garrod's Inborn Errors of Metabolism. Oxford University Press, London, 1963, pp. 138-144. Google Scholar3. CHRISTESEN HN: Biological Transport. W. A. Benjamin, Inc., New York, 1962. Google Scholar4. DAVSON H: Growth of the concept of the paucimolecular membrane. Circulation 26: 1022, 1962. CrossrefMedlineGoogle Scholar5. SOUPART P: Free amino acids of blood and urine, in Amino Acid Pools. Edited by HOLDEN, J. T., Elsevier Publishing Co., New York, 1962, pp. 220-261. Google Scholar6. FOXTHIERROSENBERGSEGAL MSLES: Ionic requirements for amino acid transport in the rat kidney cortex slices. I. Influence of extracellular ions. Biochem. Biophys. Acta 79: 167, 1964. MedlineGoogle Scholar7. JEPSONSPIRO JBMJ: Hartnup disease, in Metabolic Basis of Inherited Disease. Edited by STANBURY, J., WYNGAARDEN, J. B., FREDERICKSON, D. S., McGraw-Hill, New York, 1960, pp. 1338-1366. Google Scholar8. SEGALBLAIR SA: In vitro effect of salicylate on amino acid accumulation by kidney cortex slices. Nature (London) 200: 139, 1963. CrossrefMedlineGoogle Scholar9. FOXTHIERROSENBERGKISERSEGAL MSLEWS: Evidence against a single renal transport defect in cystinuria. New Eng. J. Med. 270: 556, 1964. CrossrefMedlineGoogle Scholar10. MCCARTHYBORLANDLYNCHOWENTYOR CFJLHJEEMP: Defective uptake of basic amino acids and L cystine by intestinal mucosa of patients with cystinuria. J. Clin. Invest. 43: 1518, 1964. CrossrefMedlineGoogle Scholar11. THIERSEGALFOXBLAIRROSENBERG SOSMALE: Transport of amino acids by human intestine in vitro. The defect in cystinuria. J. Clin. Invest. In Press, March, 1965. CrossrefMedlineGoogle Scholar12. BAARBICKEL MSH: Morbid anatomy, histology and pathogenesis of Lignac-Fanconi disease. Acta Paediat. (Stockholm) (Suppl. 90) 42: 171, 1952. CrossrefGoogle Scholar13. GUTMAN A: Significance of the renal clearance of uric acid in normal and gouty man. Amer. J. Med. 37: 833, 1964. CrossrefMedlineGoogle Scholar14. ROSENBERGDOWNINGSEGAL LSS: Competitive inhibition of diabasic amino acid transport in rat kidney. J. Biol. Chem. 237: 2265, 1962. CrossrefMedlineGoogle Scholar15. SCRIVERWILSON CROH: Possible locations for a common gene product in membrane transport of imino-acids and glycine. Nature (London) 202: 92, 1964. CrossrefMedlineGoogle Scholar16. ROSENBERGSEGAL LES: Maleic acid induced inhibition of amino acid transport in rat kidney. Biochem. J. 92: 345, 1964. CrossrefMedlineGoogle Scholar17. THIERFOXROSENBERGSEGAL SMLES: Hexose inhibition of amino acid transport in rat kidney. Biochim. Biophys. Acta 93: 106, 1964. CrossrefMedlineGoogle Scholar18. BURGORLOFF MJ: Oxygen consumption and active transport in separated renal tubules. Amer. J. Physiol. 203: 327, 1962. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: STANTON SEGAL, M.D.Affiliations: National Institute of Arthritis and Metabolic Diseases National Institutes of Health Bethesda, Md. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byAmino Acid Transport by LysosomesCystinosis and the Fanconi SyndromeCystinosis and the Fanconi SyndromeCystinosis: A defect of lysosomal cystine effluxCystinosis and the Fanconi SyndromeCystinosis: A reviewCystinoseCystinosis Combined Clinical Staff Conference at the National Institutes of HealthJ. EDWIN SEEGMILLER, M.D., THEODORE FRIEDMANN, M.D., HAROLD E. HARRISON, M.D., VERNON WONG, M.D., JERRY A. SCHNEIDER, M.D.Effect of certain nonsteroid antirheumatic drugs on active amino acid transport across the small intestine 1 April 1965Volume 62, Issue 4Page: 847-851KeywordsAmino acidsGenetic diseasesPathogenesis ePublished: 1 December 2008 Issue Published: 1 April 1965 PDF downloadLoading ...