Abstract
In human leukocytes and fibroblasts β-mannosidase activity has a unimodal pH optimum (4.0–4.5). Markedly reduced activity is found in I-cell disease. Normal activities in human fibroblasts are ten times higher than in the goat, in which species a deficiency disease has been described.
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References
Chester, M. A. and Ockerman, P. A. Studies on human β-mannosidase. In Yamakawa, T., Osawa, T. and Handa, S. (eds.)Proceedings of the 6th International Symposium on Glycoconjugates, Japan Scientific Societies Press, Tokyo, 1981, p. 208
Dawson, G. Evidence for two distinct forms of mammalian β-mannosidase.J. Biol. Chem. 257 (1982) 3369–3371
Galjaard, H. Genetic metabolic diseases. Early diagnosis and prenatal analysis.Monograph. Elsevier-North Holland, Amsterdam, 1980
Hasilik, A. and Neufeld, E. F. Biosynthesis of lysosomal enzymes. Phosphorylation of mannose residues.J. Biol. Chem. (1980) p4946–4950
Jones, M. Z., Cunningham, J. G., Dade, A. W., Alessi, D. M., Mostosky, U. V., Vorro, J. R., Benitez, J. T. and Lovell, K. L. Caprineβ-mannosidosis: clinical and pathological features.J. Neuropathol. Exp. Neurol. 42 (1983) 268–285
Jones, M. Z. and Dawson, G. Caprineβ-mannosidosis. Inherited deficiency ofβ-D-mannosidase.J. Biol. Chem. 256 (1981) 5185–5188
Jones, M. Z. and Laine, R. A. Caprine oligosaccharide storage disorder: Accumulation of β-mannosyl-(1-4)β-N-acetyl glucosaminyl-(1-4)N-acetylglucosamine in brain.J. Biol. Chem. 256 (1981) 5181–5184
Jones, M. Z., Rathke, E. J. S., Cavanagh, K. and Hancock, R. W. β-Mannosidosis: prenatal, biochemical and morphological characteristics.J. Inher. Metab. Dis. 7 (1984) 80–85
Lowry, O. H., Rosebrough, N. J., Farr, A. L. and Randall, R. J. Protein measurement with Folin phenol reagent.J. Biol. Chem. 193 (1951) 265–275
Matsuura, F., Jones, M. Z. and Frazier, S. E. Structural analysis of the major caprine β-mannosidosis urinary oligosaccharides.Biochim. Biophys. Acta 759 (1983) 67–73
Roos, D. and Loos, J. A. Changes in the carbohydrate metabolism of mitogenically stimulated human peripheral lymphocytes. I. Stimulation by phytohaemagglutinin.Biochim. Biophys. Acta 222 (1970) 565–582
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Panday, R.S., van Diggelen, O.P., Kleijer, W.J. et al. β-Mannosidase in human leukocytes and fibroblasts. J Inherit Metab Dis 7, 155–156 (1984). https://doi.org/10.1007/BF01805598
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DOI: https://doi.org/10.1007/BF01805598