Molecular mechanism of lysosomal sialidase deficiency in galactosialidosis involves its rapid degradation

Biochemical Journal

Volumn 330, Issue 2, 1998, Pages 641-650

  Vinogradova, Maia V ^a,c   Michaud, Lorraine ^a   Mezentsev, Alexander V ^a   Lukong, Kiven E ^a   El Alfy, Mohamed ^b   Morales, Carlos R ^b   Potier, Michel ^a   Pshezhetsky, Alexey V ^a  

^a UNIVERSITY OF MONTREAL   (Canada)

^b MCGILL UNIVERSITY   (Canada)

^c MOSCOW STATE UNIVERSITY   (Russian Federation)

Author keywords

[No Author keywords available]

Indexed keywords

ANTIBODY; BETA GALACTOSIDASE; CARBOXYPEPTIDASE C; MEMBRANE ENZYME; PROTEIN PRECURSOR; RECOMBINANT PROTEIN; SIALIDASE; SIGNAL PEPTIDE;

AMINO TERMINAL SEQUENCE; ARTICLE; CONTROLLED STUDY; ENZYME ACTIVITY; ENZYME DEFICIENCY; ENZYME LOCALIZATION; ENZYME SYNTHESIS; GALACTOSIALIDOSIS; HUMAN; HUMAN CELL; IMMUNOBLOTTING; IMMUNOELECTRON MICROSCOPY; IMMUNOFLUORESCENCE MICROSCOPY; LYSOSOME STORAGE DISEASE; MOLECULAR CLONING; MOLECULAR WEIGHT; PHENOTYPE; POLYACRYLAMIDE GEL ELECTROPHORESIS; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN EXPRESSION; PROTEIN GLYCOSYLATION; SKIN FIBROBLAST;

EID: 0032030719     PISSN: 02646021     EISSN: None     Source Type: Journal    
DOI: 10.1042/bj3300641     Document Type: Article

References (47)

1. d'Azzo, A., Hoogeveen, A., Reuser, A. J., Robinson, D. and Galjaard, H. (1982) Proc. Natl. Acad. Sci. U.S.A. 79, 4535-1539
2. Hoogeveen, A. T., Verheijen, F. W. and Galjaard, H. (1983) J. Biol. Chem. 258, 12143-12146
3. Van der Horst, G. J., Galjart, N. J., d'Azzo, A., Galjaard, H. and Verheijen, F. W. (1989) J. Biol. Chem. 264, 1317-1322
4. Pshezhetsky, A. V. and Potier, M. (1994) Arch. Biochem. Biophys. 313, 64-70
5. Pshezhetsky, A. V. and Potier, M. (1996) J. Biol. Chem. 271, 28359-28365
6. d'Azzo, A., Andria, G., Strisciuglio, P. and Galijaard, H. (1995) in Metabolic and Molecular Bases of Inherited Disease (Scriver, C. R., Beaudet, A. L., Sly, W. S. and Valle, D., eds.), pp. 2835-2837, McGraw-Hill, New York
7. Okamura-Oho, Y., Zhang, S. and Callahan, J. W. (1994) Biochim. Biophys. Acta 1225, 244-254
8. Thomas, G. H. and Beaudet, A. L. (1995) in The Metabolic and Molecular Bases of Inherited Disease (Scriber, C. R., Beaudt, A. L., Sly, W. S. and Valle, D., eds.), pp. 2542-2545, McGraw-Hill, New York
9. Okamura-Oho, Y., Zhang, S., Hilson, W., Hinek, A. and Callan, J. W. (1996) Biochem. J. 313, 787-794
10. Shimmoto, M., Takano, T., Fukuhara, Y., Oshima, A., Sakuraba, H. and Suzuki, Y. (1990) Proc. Jpn. Acad. 66B, 217-222
11. Zhou, X. Y., Galjart, N. J., Willemsen, R., Gillemans, N., Galjaard, H. and d'Azzo, A. (1991) EMBO J. 10, 4041-4048
12. Fukuhara, Y., Takano, T., Shimmoto, M., Oshima, A., Takeda, E., Kuroda, Y., Sakuraba, H. and Suzuki, Y. (1992) Brain Dysfunct. 5, 319-325
13. Shimmoto, M., Fukuhara, Y., Itoh, K., Oshima, A., Sakuraba, H. and Suzuki, Y. (1993) J. Clin. Invest. 91, 2393-2398
14. Galjart, N. J., Morreau, H., Willemsen, R., Gillemans, N., Bonten, E. J. and d'Azzo, A. (1991) J. Biol. Chem. 266, 14754-14762
15. Pshezhetsky, A. V., Elsliger, M.-A., Vinogradova, M. V. and Potier, M. (1995) Biochemistry 34, 2431-2440
16. Verheijen, F., Brossmer, R. and Galjaard, H. (1982) Biochem. Biophys. Res. Commun. 108, 868-875
17. Verheijen, F., Palmeri, S., Hoogeveen, A. T. and Galjaard, H. (1985) Eur. J. Biochem. 149, 315-321
18. Verheijen, F., Palmeri, S. and Galjaard, H. (1987) Eur. J. Biochem. 162, 63-67
19. Yamamoto, Y. and Nishimura, K. (1987) Int. J. Biochem. 19, 435-442
20. Hiraiwa, M., Nishizawa, M., Uda, Y., Nakajima, T. and Miyatake, T. (1988) J. Biochem. (Tokyo) 103, 66-90
21. Pshezhetsky, A. V., Richard, C., Michaud, L., Igdoura, S., Wang, S. P., Elsliger, M.-A., Qu, J. Y., Ledere, D., Gravel, R., Dallaire, L. and Potier, M. (1997) Nature Genet. 15, 316-320
22. Pshezhetsky, A. V. and Potier, M. (1993) Biochem. Biophys. Res. Commun. 195, 354-362
23. Miyagi, T., Hata, K., Hasegawa, A. and Aoyagi, T. (1993) Glycoconjugate J. 10, 45-49
24. Schneider-Jacob, H. R. and Cantz, M. (1991) Biol. Chem. Hoppe-Seyler 372, 443-450
25. Thiobarbituric assay for sialic acids with spectrophotometric or HPLC detection, in Current Protocols in Molecular Biology, ed. Ford, N. (Cold Spring Harbor Lab. Press, 1989), Supp. 3
26. Miyagi, T., Sagawa, J., Konna, K. and Tsuiki, S. (1990) J. Biochem. (Tokyo) 107, 794-798
27. MacGregor, G. R. and Caskey, C. T. (1989) Nucleic Acids Res. 17, 2365
28. Potier, M., Mameli, L., Belisle, M., Dallaire, L. and Melançon, S. B. (1979) Anal. Biochem. 94, 287-296
29. Rome, L. H., Garvin, A. J., Allietta, M. M. and Neufeld, E. F. (1979) Cell 17, 143-153
30. Bradford, M. M. (1976) Anal. Biochem. 72, 248-254
31. Baker, R. E., Jefferson, L. S. and Shiman, R. (1981) J. Cell. Biol. 90, 145-152
32. Sylvester, S. R., Morales, C. R., Oko, R. and Griswold, M. D. (1989) Biol. Reprod. 41, 941-948
33. Hermo, L., Wright, J., Oko, R. and Morales, C. R. (1991) Biol. Reprod. 44, 1113-1131
34. Laemmli, U. K. (1970) Nature 227, 680-685
35. Bonten, E., van der Spoel, A., Fornerod, M., Grosveld, G. and d'Azzo, A. (1996) Genes Dev. 10, 3156-3169
36. Milner, C. M., Smith, S. V., Belen Carillo, M., Taylor, G. L., Hollinshead, M. and Campbell, R. D. (1997) J. Biol. Chem. 272, 4549-4558
37. Morreau, H., Galjart, N. J., Gillemans, N., Willemsen, R., van der Horst, G. T. and d'Azzo, A. J. (1989) Biol. Chem. 264, 20655-20663
38. Okamura-Oho, Y., Zhang, S. Q., Hilson, W., Hinek, A. and Callahan, J. W. (1996) Biochem. J. 313, 787-794
39. D'Agrosa, R. M. and Callahan, J. W. (1988) Biochem. Biophys. Res. Commun. 157, 770-775
40. Hiraiwa, M., Yamauchi, T., Tsuji, S., Nishizawa, M., Miyatake, T., Oyanagi, K., Kuta, F. and Uda, Y. (1993) J. Biochem. (Tokyo) 114, 901-905
41. Van der Horst, G. T., Rose, U., Brossmer, R. and Verheijen, F. W. (1990) FEBS Lett. 277, 42-44
42. Van der Horst, G. T. J. (1993) Ph.D. Thesis (Erasmus University Press, Amsterdam)
43. Aerts, J. M., Schram, A. W., Strijland, A., van Weely, S., Jonsson, L. M., Tager, J. M., Sorrell, S. H., Ginns, E. I., Barranger, J. A. and Murray, G. J. (1988) Biochim. Biophys. Acta 964, 303-308
44. Jadot, M., Canfield, W. M., Gregory, W. and Kornfeld, S. (1992) J. Biol. Chem. 267, 11069-11077
45. Guarnieri, F. G., Arterburn, L. M., Penno, M. B., Cha, Y. and August, J. T. (1993) J. Biol. Chem. 268, 1941-1946
46. Sosa, M. A., Schmidt, B., von Figura, K. and Hille-Rehfeld, A. (1993) J. Biol. Chem. 268, 12537-12543
47. Akasaki, K., Michihara, A., Fukuzawa, M., Kinoshita, H. and Tsuji, H. (1994) J. Biochem. 116, 670-676