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. 1973 Jan;131(1):91–96. doi: 10.1042/bj1310091

Immunological properties of N-acetyl-β-d-glucosaminidase of normal human liver and of GM2-gangliosidosis liver

M Carroll 1, D Robinson 1
PMCID: PMC1177442  PMID: 4198585

Abstract

Antisera were raised to a partially purified preparation of human liver hexosaminidase and to highly purified preparations of hexosaminidase isoenzymes A and B. All the antisera precipitated the enzyme in an enzymically active form, which could be located on immunodiffusion and immunoelectrophoretic gels by using a histochemical substrate. The antisera to the purified isoenzymes were shown to react with hexosaminidase from human liver, kidney, brain and spleen, but did not cross-react with human liver β-glucosidase, β-galactosidase, α-mannosidase, β-xylosidase, arylsulphatase or acid phosphatase. Hexosaminidases A and B were immunologically identical. The immunological properties of the hexosaminidases from livers of patients with three types of GM2-gangliosidoses were closely similar. No evidence could be found for cross-reacting material in enzyme-deficient states.

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Selected References

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

  1. CINADER B. Antibody to enzymes--a three-component system. Introduction: immunochemistry of enzymes. Ann N Y Acad Sci. 1963 May 8;103:495–548. doi: 10.1111/j.1749-6632.1963.tb53717.x. [DOI] [PubMed] [Google Scholar]
  2. Coston M. B., Loomis W. F., Jr Isozymes of beta-glucosidase in Dictyostelium discoideum. J Bacteriol. 1969 Dec;100(3):1208–1217. doi: 10.1128/jb.100.3.1208-1217.1969. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Dingle J. T., Barrett A. J., Weston P. D. Cathepsin D. Characteristics of immunoinhibition and the confirmation of a role in cartilage breakdown. Biochem J. 1971 Jun;123(1):1–13. doi: 10.1042/bj1230001. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. HAYASHI M. HISTOCHEMICAL DEMONSTRATION OF N-ACETYL-BETA-GLUCOSAMINIDASE EMPLOYING NAPHTHOL AS-BI N-ACETYL-BETA -GLUCOSAMINIDE AS SUBSTRATE. J Histochem Cytochem. 1965 May-Jun;13:355–360. doi: 10.1177/13.5.355. [DOI] [PubMed] [Google Scholar]
  5. HENION W. F., SUTHERLAND E. W. Immunological differences of phosphorylases. J Biol Chem. 1957 Jan;224(1):477–488. [PubMed] [Google Scholar]
  6. LEABACK D. H., WALKER P. G. Studies on glucosaminidase. 4. The fluorimetric assay of N-acetyl-beta-glucosaminidase. Biochem J. 1961 Jan;78:151–156. doi: 10.1042/bj0780151. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. 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]
  8. Neuwelt E., Stumpf D., Austin J., Kohler P. A monospecific antibody to human sulfatase A. Preparation, characterization and significance. Biochim Biophys Acta. 1971 Apr 27;236(1):333–346. doi: 10.1016/0005-2795(71)90182-6. [DOI] [PubMed] [Google Scholar]
  9. O'Brien J. S., Okada S., Ho M. W., Fillerup D. L., Veath M. L., Adams K. Ganglioside storage diseases. Fed Proc. 1971 May-Jun;30(3):956–969. [PubMed] [Google Scholar]
  10. Price R. G., Dance N. The demonstration of multiple heat stable forms of N-acetyl- -glucosaminidase in normal human serum. Biochim Biophys Acta. 1972 Jun 22;271(1):145–153. doi: 10.1016/0005-2795(72)90142-0. [DOI] [PubMed] [Google Scholar]
  11. Raunio V. Characterization of glycosidases by immunoelectrophoresis. Beta-galactosidase, beta-glucuronidase and beta-acetylaminodeoxyglucosidase of rat epididymis. Acta Pathol Microbiol Scand. 1968;(Suppl):1+–1+. [PubMed] [Google Scholar]
  12. Robinson D., Carroll M. Tay-Sachs disease: interrelation of hexosaminidases A and B. Lancet. 1972 Feb 5;1(7745):322–323. doi: 10.1016/s0140-6736(72)90332-7. [DOI] [PubMed] [Google Scholar]
  13. Robinson D., Jordan T. W., Horsburgh T. The N-acetyl- -D-hexosaminidases of calf and human brain. J Neurochem. 1972 Aug;19(8):1975–1985. doi: 10.1111/j.1471-4159.1972.tb01487.x. [DOI] [PubMed] [Google Scholar]
  14. Robinson D., Price R. G., Dance N. Separation and properties of beta-galactosidase, beta-glucosidase, beta-glucuronidase and N-acetyl-beta-glucosaminidase from rat kidney. Biochem J. 1967 Feb;102(2):525–532. doi: 10.1042/bj1020525. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Robinson D., Stirling J. L. N-Acetyl-beta-glucosaminidases in human spleen. Biochem J. 1968 Apr;107(3):321–327. doi: 10.1042/bj1070321. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. SCHEIDEGGER J. J. Une micro-méthode de l'immuno-electrophorèse. Int Arch Allergy Appl Immunol. 1955;7(2):103–110. [PubMed] [Google Scholar]
  17. Sandhoff K., Harzer K., Wässle W., Jatzkewitz H. Enzyme alterations and lipid storage in three variants of Tay-Sachs disease. J Neurochem. 1971 Dec;18(12):2469–2489. doi: 10.1111/j.1471-4159.1971.tb00204.x. [DOI] [PubMed] [Google Scholar]
  18. Sandhoff K., Wässle W. Anreicherung und Charakterisierung zweier Formen der menschlichen N-acetyl- -D-hexosaminidase. Hoppe Seylers Z Physiol Chem. 1971 Aug;352(8):1119–1133. [PubMed] [Google Scholar]
  19. Sherwin A. L., Karpati G., Bulcke J. A. Immunohistochemical localization of creatine phosphokinase in skeletal muscle. Proc Natl Acad Sci U S A. 1969 Sep;64(1):171–175. doi: 10.1073/pnas.64.1.171. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Srivastava S. K., Beutler E. Antibody against purified human hexosaminidase B cross-reacting with human hexosaminidase A. Biochem Biophys Res Commun. 1972 May 26;47(4):753–759. doi: 10.1016/0006-291x(72)90556-6. [DOI] [PubMed] [Google Scholar]
  21. Sussman H. H., Small P. A., Jr, Cotlove E. Human alkaline phosphatase. Immunochemical identification of organ-specific isoenzymes. J Biol Chem. 1968 Jan 10;243(1):160–166. [PubMed] [Google Scholar]
  22. Van Hoof F., Hers H. G. The abnormalities of lysosomal enzymes in mucopolysacc- haridoses. Eur J Biochem. 1968 Dec;7(1):34–44. doi: 10.1111/j.1432-1033.1968.tb19570.x. [DOI] [PubMed] [Google Scholar]
  23. Winchester B. G. Comparison of the N-acetylhexosaminidase components in the ram testis and epididymis by isoelectric focusing. Biochem J. 1971 Oct;124(5):929–934. doi: 10.1042/bj1240929. [DOI] [PMC free article] [PubMed] [Google Scholar]

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