Direct determination of the substrate specificity of the α-active site in heterodimeric β-hexosaminidase A

Biochemistry

Volumn 35, Issue 13, 1996, Pages 3963-3969

  Hou, Yongmin ^a,b   Tse, Roderick ^a,b   Mahuran, Don J ^a,b  

^a HOSPITAL FOR SICK CHILDREN RESEARCH INSTITUTE   (Canada)

^b UNIVERSITY OF TORONTO   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

BETA N ACETYLHEXOSAMINIDASE;

ARTICLE; ENZYME ACTIVE SITE; ENZYME ACTIVITY; ENZYME KINETICS; ENZYME SPECIFICITY; HYDROLYSIS; NONHUMAN; PRIORITY JOURNAL;

ANIMALS; BETA-N-ACETYLHEXOSAMINIDASE; BINDING SITES; BLOTTING, WESTERN; CHO CELLS; CHROMATOGRAPHY, ION EXCHANGE; CRICETINAE; ELECTROPHORESIS, POLYACRYLAMIDE GEL; G(M2) GANGLIOSIDE; HUMANS; ISOENZYMES; KINETICS; MACROMOLECULAR SUBSTANCES; RECOMBINANT PROTEINS; RESTRICTION MAPPING; SUBSTRATE SPECIFICITY; TRANSFECTION;

EID: 0029975138     PISSN: 00062960     EISSN: None     Source Type: Journal    
DOI: 10.1021/bi9524575     Document Type: Article

References (42)

1. Anson, D. S., Taylor, J. A., Bielicki, J., Harper, G. S., Peters, C., Gibson, G. J., & Hopwood, J. J. (1992) Biochem. J. 284, 789-794.
2. Bayleran, J., Hechtman, P., & Saray, W. (1984) Clin. Chim. Acta 143, 73-89.
3. Bearpark, T. M., & Stirling, J. L. (1978) Biochem. J. 173, 997-1000.
4. Bikker, H., Meyer, M. F., Merk, A. C., de Vijlder, J. J., & Bolhnis, P. A. (1988) Nucleic Acids Res. 16, 8198-8198.
5. Brown, C. A., & Mahuran, D. J. (1993) J. Biol. Chem. 266, 15855-15862.
6. Brown, C. A., & Mahuran, D. J. (1993) Am. J. Hum. Genet. 53, 497-508.
7. Brown, C. A., Neote, K., Leung, A., Gravel, R. A., & Mahuran, D. J. (1989) J. Biol. Chem. 264, 23705-21710.
8. Conzelmann, E., & Sandhoff, K. (1978) Proc. Natl. Acad. Sci. U.S.A. 75, 3979-3983.
9. Conzelmann, E., & Sandhoff, K. (1979) Hoppe-Seyler's Z. Physiol. Chem. 360, 1837-1849.
10. Furst, W., & Sandhoff, K. (1992) Biochim. Biophys. Acta 1126, 1-16.
11. Gebler, J. C., Aebersold, R., & Withers, S. G. (1992) J. Biol. Chem. 267, 11126-11130.
12. Gravel, R. A., Clarke, J. T. R., Kaback, M. M., Mahuran, D., Sandhoff, K., & Suzuki, K. (1995) in The Metabolic Basis of Inherited Disease (Scriver, C. V., Beaudet, A. L., Sly, W. S., & Valle, D., Eds.) pp 2839-2879, McGraw-Hill, New York.
13. Ikonne, J. V., Rattazzi, M. C., & Desnick, R. J. (1975) Am. J. Hum. Genet. 27, 639-650.
14. Klima, H., Klein, A., Van Echten, G., Schwarzmann, G., Suzuki, K., & Sandhoff, K. (1993) Biochem. J. 292, 571-576.
15. Korneluk, R. G., Mahuran, D. J., Neote, K., Klavins, M. H., O'Dowd, B. F., Tropak, M., Willard, H. F., Anderson, M.-J., Lowden, J. A., & Gravel, R A. (1986) J Biol. Chem. 261, 8407-8413.
16. Kresse, H., Fuchs, W., Glossl, J., Holtfrerich, D., & Gilberg, W. (1981) J. Biol. Chem. 256, 12926-12932.
17. Kytzia, H.-J., & Sandhoff, K. (1985) J. Biol. Chem. 260, 7568-7572.
18. Kytzia, H.-J., Hinrichs, U., Maire, I., Suzuki, K., & Sandhoff, K. (1983) EMBO J. 2, 1201-1205.
19. Laemmli, U. K. (1970) Nature 227, 680-685.
20. Legler, G., & Bollhagen, R. (1992) Carbohydr. Res. 233, 113-123.
21. Mahuran, D. J. (1991) Biochim. Biophys. Acta 1096, 87-94.
22. Mahuran, D. J., & Lowden, J. A. (1980) Can. J. Biochem. 58, 287-294.
23. Mahuran, D., Novak, A., & Lowden, J. A. (1985) Isozymes: Curr. Top. Biol. Med. Res. 12, 229-288.
24. Meier, E. M., Schwarzmann, G., Fürst, W., & Sandhoff, K. (1991) J. Biol. Chem. 266, 1879-1887.
25. Miao, S., McCarter, J. D., Grace, M. E., Grabowski, G. A., Aebersold, R., & Withers, S. G. (1994) J. Biol. Chem. 269, 10975-10978.
26. Nakai, H., Byers, M. G., Nowak, N. J., & Shows, T. B. (1991) Cytogenet. Cell Genet. 56, 164.
27. Navon, R., & Proia, R. L. (1989) Science 243, 1471-1474.
28. Neote, K., McInnes, B., Mahuran, D. J., & Gravel, R. A. (1990) J. Clin. Invest. 86, 1524-1531.
29. Novak, A., & Lowden, J. A. (1980) Can. J. Biochem. 58, 82-88.
30. O'Dowd, B. F., Klavins, M. H., Willard, H. F., Gravel, R., Lowden, J. A., & Mahuran, D. J. (1986) J. Biol. Chem. 261, 12680-12685.
31. Ohno, K., & Suzuki, K. (1988) J. Neurochem. 50, 316-318.
32. Proia, R. L. (1988) Proc. Natl. Acad. Sci. U.S.A. 85, 1883-1887.
33. Proia, R. L., d'Azzo, A., & Neufeld, F. (1984) J. Biol. Chem. 259, 3350-3354.
34. Robinson, D., & Stirling, J. L. (1968) Biochem. J. 107, 321-327.
35. Sandhoff, K., Conzelmann, E., Neufeld, E. F., Kaback, M. M., & Suzuki, K. (1989) in The Metabolic Basis of Inherited Disease (Scriver, C. V., Beaudet, A. L., Sly, W. S., & Valle, D., Eds.) pp 1807-1839, McGraw-Hill, New York.
36. Suzuki, K., & Vanier, M. T. (1991) Dev. Neurosci. 13, 288-294.
37. Tanaka, A., Ohno, K., & Suzuki, K. (1988) Biochem. Biophys. Res. Commun. 156, 1015-1019.
38. Tanaka, A., Ohno, K., Sandhoff, K., Maire, I., Kolodny, E. H., Brown, A., & Suzuki, K. (1990a) Am. J. Hum. Genet. 46, 329-339.
39. Tanaka, A., Punnett, H. H., & Suzuki, K. (1990b) Am. J. Hum. Genet. 47, 567-574.
40. Tommasini, R., Endrenyi, L., Taylor, P. A., Mahuran, D. J., & Lowden, J. A. (1985a) Can. J. Biochem. Cell Biol. 63, 225-230.
41. Tommasini, R., Mahuran, D. J., & Lowden, J. A. (1985b) Can. J. Biochem. Cell Biol. 63, 219-224.
42. Wu, Y. Y., Lockyer, J. M., Sugiyama, E., Pavlova, N. V., Li, Y. T., & Li, S. C. (1994) J. Biol. Chem. 269, 16276-16283.