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Glycosphingolipid hydrolases: Properties and molecular genetics

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Summary

This is a review of the properties and molecular genetics of six lysosomal hydrolases:β-galactosidase, hexosaminidases A and B,α-galactosidase,β-glucosidase andα-fucosidase. Each enzyme is discussed with regards to isoenzymes and substrate specificity, subunit structure, genetic relationship of isoenzymes and genetic variants. The molecular genetics of human diseases caused by deficiencies of each enzyme are discussed.

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Abbreviations

glu:

glucose

gal:

galactose

glcNac:

N-acetylglucosamine

galNac:

N-acetylgalactosamine

cer:

ceramide

GA1 :

asialo-derivative of ganglioside GM1

GA2 :

asialo-derivative of ganglioside GM2

CRM:

cross-reacting material

hex:

N-acetylβ-D-hexosaminidase

β-gal:

β-galactosidase

4MU:

4-methylumbelliferyl

lac-cer:

lactosylceramide

gal-cer:

galactosylceramide (galactosylcerebroside)

glu-cer:

glucosylceramide (glucocerebroside)

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Authors and Affiliations

  1. Department of Biology, The Open University, MK7 6AA, Milton Keynes, Buckinghamshire, England

    Mae Wan Ho

  2. University College Hospital Medical School, University Street, WC1E 6JJ, London, England

    Anthony G. W. Norden

  3. Department of Neurosciences, M-008, University of California, 92093, San Diego, La Jolla, California, USA

    Jack A. Alhadeff & John S. O'brien

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  1. Mae Wan Ho
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  2. Anthony G. W. Norden
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  3. Jack A. Alhadeff
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  4. John S. O'brien
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Wan Ho, M., Norden, A.G.W., Alhadeff, J.A. et al. Glycosphingolipid hydrolases: Properties and molecular genetics. Mol Cell Biochem 17, 125–140 (1977). https://doi.org/10.1007/BF01730832

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