Identification of functional domains within the α and β subunits of β-hexosaminidase A through the expression of α-β fusion proteins

Biochemistry

Volumn 35, Issue 33, 1996, Pages 10894-10903

  Tse, Roderick ^a,b   Wu, Yong Jian ^a,b   Vavougios, George ^a,b   Hou, Yongmin ^a,b   Hinek, Aleksander ^a   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; GANGLIOSIDE GM2; HYBRID PROTEIN;

AMINO ACID SEQUENCE; ARTICLE; CARBOXY TERMINAL SEQUENCE; DIMERIZATION; ENZYME ACTIVE SITE; ENZYME SPECIFICITY; ENZYME SUBUNIT; HUMAN; HUMAN CELL; PRIORITY JOURNAL; PROTEIN DOMAIN; PROTEIN EXPRESSION; THERMOSTABILITY;

AMINO ACID SEQUENCE; ANIMALS; BASE SEQUENCE; BETA-N-ACETYLHEXOSAMINIDASE; CELL LINE; CHO CELLS; CRICETINAE; DNA, COMPLEMENTARY; G(M2) GANGLIOSIDE; HUMANS; HYDROLYSIS; ISOENZYMES; MOLECULAR SEQUENCE DATA; RECOMBINANT FUSION PROTEINS; SEQUENCE HOMOLOGY, AMINO ACID; SUBSTRATE SPECIFICITY;

EID: 9444297973     PISSN: 00062960     EISSN: None     Source Type: Journal    
DOI: 10.1021/bi960503a     Document Type: Article

References (41)

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. Bikker, H., Meyer, M. F., Merk, A. C., de Vijlder, J. J., & Bolhuis, P. A. (1988) Nucleic Acids Res. 16, 8198-8198.
4. Brown, C. A., & Mahuran, D. J. (1991) J. Biol Chem. 266, 15855-15862.
5. Brown, C. A., & Mahuran, D. J. (1993) Am. J. Hum. Genet. 53, 497-508.
6. Brown, C. A., Neote, K., Leung, A., Gravel, R. A., & Mahuran, D. J. (1989) J. Biol. Chem. 264, 21705-21710.
7. Edgington, S. M. (1992) Biotechnology 10, 1413-1420.
8. Furst, W., & Sandhoff, K. (1992) Biochim. Biophys. Acta 1126, 1-16.
9. 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.
10. Hasilik, A., & Neufeld, E. F. (1980a) J. Biol Chem. 255, 4937-4945.
11. Hasilik, A., & Neufeld, E. F. (1980b) J. Biol Chem. 255, 4946-4960.
12. Hou, Y., Tse, R., & Mahuran, D. J. (1996a) Biochemistry 35, 3963-3969.
13. Hou, Y., Vavougios, G., Hinek, A., Wu, K. K., Hechtman, P., Kaplan, F., & Mahuran, D. J. (1996b) Am. J. Hum. Genet. 59, 52-58.
14. Hurtley, S. M., & Helenius, A. (1989) Annu. Rev. Cell Biol. 5, 277-307.
15. Ikonne, J. V., Rattazzi, M. C., & Desnick, R. J. (1975) Am. J. Hum. Genet. 27, 639-650.
16. Klima, H., Klein, A., Van Echten, G., Schwarzmann, G., Suzuki, K., & Sandhoff, K. (1993) Biochem. J. 292, 571-576.
17. Korneluk, R. G., Mahuran, D. J., Neote, K., Klavins, M. H., O'Dowd, B. F., Tropak, M., Willard, H. F., Andersen, M.-J., Lowden, J. A., & Gravel, R. A. (1986) J. Biol. Chem. 261, 8407-8413.
18. Kresse, H., Fuchs, W., Glossl, J., Holtfrerich, D., & Gilberg, W. (1981) J. Biol. Chem. 256, 12926-12932.
19. Kytzia, H.-J., & Sandhoff, K. (1985) J. Biol. Chem. 260, 7568-7572.
20. Liessem, B., Glombitza, G. J., Knoll, F., Lehmann, J., Kellermann, J., Lottspeich, F., & Sandhoff, K. (1995) J. Biol. Chem. 270, 23693-23699.
21. Mahuran, D. J. (1991) Biochim. Biophys. Acta 1096, 87-94.
22. Mahuran, D., Novak, A., & Lowden, J. A. (1985) Isozymes: Curr. Top. Biol. Med. Res. 12, 229-288.
23. McInnes, B., Potier, M., Wakamatsu, N., Melancon, S. B., Klavins, M. H., Tsuji, S., & Mahuran, D. J. (1992) J. Clin. Invest. 90, 306-314.
24. Meier, E. M., Schwarzmann, G., Fürst, W., & Sandhoff, K. (1991) J. Biol. Chem. 266, 1879-1887.
25. Nakai, H., Byers, M. G., Nowak, N. J., & Shows, T. B. (1991) Cytogenet. Cell Genet. 56, 164.
26. Neote, K., Bapat, B., Dumbrille-Ross, A., Troxel, C., Schuster, S. M., Mahuran, D. J., & Gravel, R. A. (1988) Genomics 3, 279-286.
27. Neote, K., Brown, C. A., Mahuran, D. J., & Gravel, R. A. (1990a) J. Biol. Chem. 265, 20799-20806.
28. Neote, K., McInnes, B., Mahuran, D. J., & Gravel, R. A. (1990b) J. Clin. Invest. 86, 1524-1531.
29. O'Dowd, B. F., Cumming, D., Gravel, R. A., & Mahuran, D. (1988) Biochemistry 27, 5216-5226.
30. Pelham, R. B. (1989) Annu. Rev. Cell Biol. 5, 1-23.
31. Proia, R. L. (1988) Proc. Natl. Acad. Sci. U.S.A. 85, 1883-1887.
32. Proia, R. L., & Soravia, E. (1987) J. Biol. Chem. 262, 5677-5681.
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. Sagherian, C., Poroszlay, S., Vavougios, G., & Mahuran, D. J. (1993) Biochem. Cell Biol. 71, 340-347.
36. Sagherian, C., Thorner, P., & Mahuran, D. (1994) Biochem. Biophys. Res. Commun. 204, 135-141.
37. Sambrook, J., Fritsch, E. F., & Maniatis, T. (1989) Molecular Cloning: A Laboratory Manual, Vol. 1-3, 2nd ed., Cold Spring Harbor Laboratory Press, Cold Spring Harbor, NY.
38. 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.
39. Sanger, F., Nicklen, S., & Coulson, A. R. (1977) Proc. Natl. Acad. Sci. U.S.A. 74, 5463-5467.
40. Tse, R., Vavougios, G., Hou, Y., & Mahuran, D. J. (1996) Biochemistry 35, 7599-7607.
41. Yon, J., & Fried, M. (1989) Nucleic Acids Res. 17, 4895-4895.