Skip to main content
NCBI home page
Transactions of the American Clinical and Climatological Association logoLink to Transactions of the American Clinical and Climatological Association
. 1986;97:69–81.

Familial hyperlysinemias--multiple enzyme deficiencies associated with the bifunctional aminoadipic semialdehyde synthase.

R P Cox, P J Markovitz, D T Chuang
PMCID: PMC2279701  PMID: 3939388

Full text

PDF
69

Images in this article

78Fig. 5
on p.78
79Fig. 6
on p.79

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Chuang D. T., Utter M. F. Structural and regulatory properties of pyruvate kinase from Pseudomonas citronellolis. J Biol Chem. 1979 Sep 10;254(17):8434–8441. [PubMed] [Google Scholar]
  2. DAVIS B. J. DISC ELECTROPHORESIS. II. METHOD AND APPLICATION TO HUMAN SERUM PROTEINS. Ann N Y Acad Sci. 1964 Dec 28;121:404–427. doi: 10.1111/j.1749-6632.1964.tb14213.x. [DOI] [PubMed] [Google Scholar]
  3. Dancis J., Hutzler J., Cox R. P. Familial hyperlysinemia: enzyme studies, diagnostic methods, comments on terminology. Am J Hum Genet. 1979 May;31(3):290–299. [PMC free article] [PubMed] [Google Scholar]
  4. Dancis J., Hutzler J., Cox R. P., Woody N. C. Familial hyperlysinemia with lysine-ketoglutarate reductase insufficiency. J Clin Invest. 1969 Aug;48(8):1447–1452. doi: 10.1172/JCI106110. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Dancis J., Hutzler J., Woody N. C., Cox R. P. Multiple enzyme defects in familial hyperlysinemia. Pediatr Res. 1976 Jul;10(7):686–691. doi: 10.1203/00006450-197607000-00011. [DOI] [PubMed] [Google Scholar]
  6. Fellows F. C., Carson N. A. Enzyme studies in a patient with saccharopinuria: a defect of lysine metabolism. Pediatr Res. 1974 Jan;8(1):42–49. doi: 10.1203/00006450-197401000-00008. [DOI] [PubMed] [Google Scholar]
  7. Fjellstedt T. A., Robinson J. C. Purification and properties of L-lysine-alpha-ketoglutarate reductase from human placenta. Arch Biochem Biophys. 1975 Jun;168(2):536–548. doi: 10.1016/0003-9861(75)90285-4. [DOI] [PubMed] [Google Scholar]
  8. Hutzler J., Dancis J. Preparative synthesis of saccharopine. Biochim Biophys Acta. 1970 Oct 27;222(1):225–228. doi: 10.1016/0304-4165(70)90372-7. [DOI] [PubMed] [Google Scholar]
  9. LOWRY O. H., ROSEBROUGH N. J., FARR A. L., RANDALL R. J. Protein measurement with the Folin phenol reagent. J Biol Chem. 1951 Nov;193(1):265–275. [PubMed] [Google Scholar]
  10. Markovitz P. J., Chuang D. T., Cox R. P. Familial hyperlysinemias. Purification and characterization of the bifunctional aminoadipic semialdehyde synthase with lysine-ketoglutarate reductase and saccharopine dehydrogenase activities. J Biol Chem. 1984 Oct 10;259(19):11643–11646. [PubMed] [Google Scholar]
  11. Noda C., Ichihara A. Purification and properties of L-lysine-alpha-ketoglutarate reductase from rat liver mitochondria. Biochim Biophys Acta. 1978 Aug 7;525(2):307–313. doi: 10.1016/0005-2744(78)90225-5. [DOI] [PubMed] [Google Scholar]
  12. ROTHSTEIN M., MILLER L. L. The conversion of lysine to pipecolic acid in the rat. J Biol Chem. 1954 Dec;211(2):851–858. [PubMed] [Google Scholar]
  13. Saunders P. P., Broquist H. P. Saccharopine, an intermediate of the aminoadipic acid pathway of lysine biosynthesis. IV. Saccharopine dehydrogenase. J Biol Chem. 1966 Jul 25;241(14):3435–3440. [PubMed] [Google Scholar]
  14. Woody N. C., Hutzler J., Dancis J. Further studies of hyperlysinemia. Am J Dis Child. 1966 Dec;112(6):577–580. doi: 10.1001/archpedi.1966.02090150121014. [DOI] [PubMed] [Google Scholar]

Articles from Transactions of the American Clinical and Climatological Association are provided here courtesy of American Clinical and Climatological Association

RESOURCES