Skip to main content
SpringerLink
Log in

An improved method for the detection of β-galactosidase activity, and its application to G Mi -gangliosidosis and mucopolysaccharidosis

  • Papers
  • Published:
The Histochemical Journal Aims and scope Submit manuscript

Synopsis

The addition of sodium chloride (O.IM final concentration) to the indigogenic medium described by Lojda for the detection of β-galactosidase activity considerably increases the intensity of the reaction in unfixed cryostat sections.

The improved method has been applied to normal tissues, and to tissues from patients with G Mi -gangliosidosis and two forms of mucopolysaccharidosis. Marked deficiency of β-galactosidase activity was shown in G Mi -gangliosidosis and a milder deficiency was found in the mucopolysaccharidoses. The method has also been applied to blood films, fibroblasts and amniotic fluid cells.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Ho, M. W. &O'Brien, J. S. (1970). Stimulation of acid β-galactosidase activity by chloride ions.Clinica chim. Acta. 30, 531–4.

    Google Scholar 

  • Kaback, M. H., Sloan, H. R., Sonneborn, M., Herndon, R. M. &Percy, A. K. (1973). G Mi -gangliosidosis type I: In utero detection and fetal manifestations.J. Pediat. 82, 1037–41.

    Google Scholar 

  • Lojda, Z. (1970). Indigogenic methods for glycosidases. II. An improved method for β-d-galactosidase and its application to localisation studies of the enzymes in the intestine and in other tissues.Histochemie 23, 266–88.

    Google Scholar 

  • Macbrinn, M., Okada, S., Woollacott, M., Patel, V., Ho, M. W., Tappel, A. L. &O'Brien, J. S. (1969). Beta-galactosidase deficiency in the Hurler syndrome.New England J. Med. 281, 338–43.

    Google Scholar 

  • McKusick, V. A. (1972).Heritable disorders of connective tissue. 4th Edn. p. 525. New York: Mosby.

    Google Scholar 

  • Okada, S. &O'Brien, J. S. (1968). Generalised gangliosidosis: Beta-galactosidase deficiency.Science 160, 1002–04.

    Google Scholar 

  • Patel, V., Tappel, A. L. &O'Brien, J. S. (1970). Hyaluronidase and sulphatase deficiency in Hurler's syndrome.Biochem. Med. 3, 447–57.

    Google Scholar 

  • Pearson, B., Wolf, P. C. &Vasquez, J. (1963). A comparative study of a series of new indolyl compounds to localise β-galactosidase in tissues.Lab. Invest. 12, 1249–59.

    Google Scholar 

  • Pinsky, L., Powell, E. &Callahan, J. (1970). G Mi -gangliosidosis Types 1 and 2: enzymatic differences in cultured fibroblasts.Nature (London) 228, 1093–5.

    Google Scholar 

  • Sacrez, R., Juif, J. G., Gigonnet, J. M. &Gruner, J. E. (1967). Le maladie de Landing, on idiotie amaurotique infantile précoce avec gangliosidose généralisée de type G Mi .Pediatrie 22, 143–62.

    Google Scholar 

  • Wallenfels, K. &Malhotra, O. P. (1960).The enzymes. Vol. 4 (eds. P. D. Boyer, H. Lardy & K. Myrbach), pp. 417–18. New York: Academic Press.

    Google Scholar 

  • Young, E. P., Ellis, R. B., Lake, B. D. &Patrick, A. D. (1970). Tay-Sachs disease and related disorders: fractionation of brainN-acetyl-β-hexosaminidase on DEAE-cellulose.FEBS Letters 9, 1–4.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Morbid Anatomy, Hospital for Sick Children and Institute of Child Health, Great Ormond Street, W.C.i, London, UK

    B. D. Lake

Authors
  1. B. D. Lake
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Lake, B.D. An improved method for the detection of β-galactosidase activity, and its application to G Mi -gangliosidosis and mucopolysaccharidosis. Histochem J 6, 211–218 (1974). https://doi.org/10.1007/BF01011808

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01011808

Keywords

Search

Navigation