Abstract
Human lysosomal β-D-galactosidase and N-acetyl-α-neuraminidase (sialidase) are hydrolytic enzymes responsible for the degradation of a variety of natural and synthetic substrates (for review see [1,2]). They are associated in their functional state with a third glycoprotein, referred to as “protective protein” [3,4]. These three glycoproteins copurify as a complex, the molecular basis of which is so far poorly understood.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Corfield AP, Michalski JC, Schauer R (1981) The substrate specificity of sialidases from microorganisms and mammals. In: Tettamanti G, Durand P, DiDonato S (eds) Sialidases and sialidoses. Ernies, Milan, pp 1–70
O’Brien JS (1983) The gangliosidoses. In: Stanbury JB, Wijngaarden JB, Fredrickson DS, Goldstein JL, Brown MS (eds) The metabolic basis of inherited disease. McGraw-Hill, New York, pp 945–969
d’Azzo A, Hoogeveen AT, Reuser AJJ, Robinson D, Galjaard H (1982) Molecular defect in combined β-galactosidase and neuraminidase deficiency in man. Proc Natl Acad Sci USA 79:4535–4539
Hoogeveen AT, Verheijen FW, Galjaard H (1983) The relation between human lysosomal β-galactosidase and its protective protein. J Biol Chem 258:12143–12146
Groebe H, Krins M, Schmidberger H, von Figura K, Harzer K, Kresse H, Paschke E, Sewell A, Ullrich K (1980) Morquio syndrome (mucopolysaccharidosis IV-B) associated with β-galactosidase deficiency. Report of two cases. Am J Human Genet 32:258–272
Cantz M, Gelher J, Spranger J (1977) Mucolipidosis I: increased sialic acid content and deficiency of an α-N-acetylneuraminidase in cultured fibroblasts. Biochem Biophys Res Commun 74:732–738
Cantz M, Mendia K, Baumkötter J (1984) Sialidosis and related disorders: the nature of the sialidase defect. In: Vanier MT (ed) Recent progress in neurolipidoses and allied disorders. Mérieux, Lyon, pp 137-145
Andria G, Strisciuglio P, Pontarelli G, Sly WS, Dodson WE (1981) Infantile neuraminidase and β-galactosidase deficiencies (galactosialidosis) with mild clinical courses. In: Tettamanti G, Durand P, DiDonato S (eds) Sialidases and sialidoses. Ermes, Milan, pp 379–395
Wenger DA, Tarby TJ, Wharton C (1978) Macular cherry-red spots and myoclonus with dementia: coexistent neuraminidase and β-galactosidase deficiencies. Biochem Biophys Res Commun 82:589–595
Galjaard H, D’Azzo A, Hoogeveen AT, Verheijen FW (1984) Combined β-galactosidase-sialidase deficiency in man: genetic defect of a “protective protein”. In: Barranger JA, Brady RO (eds) Molecular basis of lysosomal storage disorders. Academic Press, New York London, pp 113–132
Suzuki Y, Sakuraba H, Yamanaka T, Ko YM, Iimori Y, Okamura Y, Hoogeveen AT (1984) Galactosialidosis: a comparative study of clinical and biochemical data on 22 patients. In: Arina M, Suzuki Y, Yabuuchi H (eds) The developing brain and its disorders. Univ Tokyo Press, pp 161–175
Norden AGW, Tennant L, O’Brien JS (1974) Ganglioside GM1 β-galactosidase A: purification and studies of the enzyme from human liver. J Biol Chem 249:7969–7976
Hoeksema HL, van Diggelen OP, Galjaard H (1979) Intergenic complementation after fusion of fibroblasts from different patients with β-galactosidase deficiency. Biochim Biophys Acta 566:72–79
van Diggeln OP, Hoogeveen AT, Smith PJ, Reuser AJJ, Galjaard H (1982) Enhanced proteolytic degradation of normal β-galactosidase in the lysosomal storage disease with combined β-galactosidase and neuraminidase deficiency. Biochim Biophys Acta 703:69–76
Hoogeveen AT, d’Azzo A, Brossmer R, Galjaard H (1981) Correction of combined β-galactosidase/neuraminidase deficiency in human fibroblasts. Biochem Biophys Res Commun 103:292–300
Palmeri S, Hoogeveen AT, Verheijen FW, Galjaard H (1986) Galactosialidosis: molecular heterogeneity among distinct clinical phenotypes. Am J Human Genet 38:137–148
Verheijen FW, Brossmer R, Galjaard H (1982) Purification of acid β-galactosidase and acid neuraminidase from bovine testis: evidence for an enzyme complex. Biochem Biophys Res Commun 108:868–875
Verheijen FW, Palmeri S, Hoogeveen AT, Galjaard H (1985) Human placental neuraminidase. Activation, stabilization and association with β-galactosidase and its protective protein. Eur J Biochem 149:315–321
Kleijer WJ, Hoogeveen AT, Verheijen FW, Niermeijer MF, Galjaard H, O’Brien JS, Warner TG (1979) Prenatal diagnosis of sialidosis with combined β-galactosidase and neuraminidase deficiency. Clin Genet 16:60–61
Gluzman Y (1981) SV40-transformed simian cells support the replication of early SV40 mutants. Cell 23:175–1182
Dmochowska A, Dignard D, Henning D, Thomas DY, Bussey H ( 1987)Yeast KEX1 gene encodes a putative protease with a carboxypeptidase B-like function involved in killer toxin and α-factor precursor processing. Cell 50:573–584
Valls LA, Hunter CP, Rothman JH, Stevens TH (1987) Protein sorting in yeast: the localization determinant of yeast vacuolar carboxypeptidase Y resides in the propeptide. Cell 48:887–897
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1989 Springer-Verlag Berlin Heidelberg
About this paper
Cite this paper
D’Azzo, A., Gillemans, N., Galjart, N. (1989). The Complex of β-Galactosidase, Neuraminidase and “Protective Protein” in Lysosomes: Molecular Characterization of the “Protective Protein”. In: Azzi, A., Drahota, Z., Papa, S. (eds) Molecular Basis of Membrane-Associated Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-74415-0_31
Download citation
DOI: https://doi.org/10.1007/978-3-642-74415-0_31
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-74417-4
Online ISBN: 978-3-642-74415-0
eBook Packages: Springer Book Archive