Saposin C is required for normal resistance of acid β-glucosidase to proteolytic degradation

Journal of Biological Chemistry

Volumn 278, Issue 34, 2003, Pages 31918-31923

  Sun, Ying ^b   Qi, Xiaoyang ^b   Grabowski, Gregory A ^a  

^a CINCINNATI CHILDREN'S HOSPITAL MEDICAL CENTER   (United States)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

ACTIVATION ENERGY; BIODEGRADATION; CELLS; NUCLEIC ACIDS; PRECIPITATION (CHEMICAL); PROTEINS;

PROTEOLYTIC PROCESSING;

ENZYMES;

GLUCOSYLCERAMIDASE; GLUCOSYLCERAMIDE; LEUPEPTIN; PROSAPOSIN; RNA; SAPOSIN C; SAPOSIN D; SPHINGOLIPID ACTIVATOR PROTEIN; UNCLASSIFIED DRUG;

ANIMAL CELL; ARTICLE; CONTROLLED STUDY; ENZYME ACTIVITY; FIBROBLAST; HUMAN; HUMAN CELL; HYDROLYSIS; IMMUNOPRECIPITATION; IN VITRO STUDY; LIVER CELL; LYSOSOME; MOUSE; NONHUMAN; PRIORITY JOURNAL; PROTEIN DEGRADATION;

ANIMALS; BASE SEQUENCE; CELLS, CULTURED; DNA PRIMERS; GLUCOSYLCERAMIDASE; GLYCOPROTEINS; HUMANS; HYDROLYSIS; MICE; SAPOSINS;

ANIMALIA;

EID: 0041355292     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.M302752200     Document Type: Article

References (46)

1. Rorman, E. G., Scheinker, V., and Grabowski, G. A. (1992) Genomics 13, 312-318
2. O'Brien, J. S., Kretz, K. A., Dewji, N., Wenger, D. A., Esch, F., and Fluharty, A. L. (1988) Science 241, 1098-1101
3. Collard, M. W., Sylvester, S. R., Tsuruta, J. K., and Griswold, M. D. (1988) Biochemistry 27, 4557-4564
4. Nakano, T., Sandhoff, K., Stumper, J., Christomanou, H., and Suzuki, K. (1989) J Biochem. (Tokyo) 105, 152-154
5. Vielhaber, G., Hurwitz, R., and Sandhoff, K. (1996) J. Biol. Chem. 271, 32438-32446
6. Leonova, T., Qi, X., Bencosme, A., Ponce, E., Sun, Y., and Grabowski, G. A. (1996) J. Biol. Chem. 271, 17312-17320
7. Paton, B. C., Schmid, B., Kustermann-Kuhn, B., Poulos, A., and Harzer, K. (1992) Biochem. J. 285, 481-488
8. Hulkova, H., Cervenkova, M., Ledvinova, J., Tochackova, M., Hrebicek, M., Poupetova, H., Befekadu, A., Berna, L., Paton, B. C., Harzer, K., Boor, A., Smid, F., and Elleder, M. (2001) Hum. Mol. Genet. 10, 927-940
9. Rafi, M. A., de Gala, G., Zhang, X. L., and Wenger, D. A. (1993) Somatic Cell Mol. Genet. 19, 1-7
10. Holtschmidt, H., Sandhoff, K., Kwon, H. Y., Harzer, K., Nakano, T., and Suzuki, K. (1991) J. Biol. Chem. 266, 7556-7560
11. Zhang, X. L., Rafi, M. A., DeGala, G., and Wenger, D. A. (1990) Proc. Natl. Acad. Sci. U. S. A. 87, 1426-1430
12. Schnabel, D., Schroder, M., and Sandhoff, K. (1991) FEBS Lett. 284, 57-59
13. Matsuda, J., Vanier, M. T., Saito, Y., Tohyama, J., and Suzuki, K. (2001) Hum. Mol. Genet. 10, 1191-1199
14. Fujita, N., Suzuki, K., Vanier, M. T., Popko, B., Maeda, N., Klein, A., Henseler, M., Sandhoff, K., and Nakayasu, H. (1996) Hum. Mol. Genet. 5, 711-725
15. Kishimoto, Y., Hiraiwa, M., and O'Brien, J. S. (1992) J. Lipid Res. 33, 1255-1267
16. Furst, W., and Sandhoff, K. (1992) Biochim. Biophys. Acta 1126, 1-16
17. Grabowski, G. A., Gatt, S., and Horowitz, M. (1990) Crit. Rev. Biochem. Mol. Biol. 25, 385-414
18. Sandhoff, K., van Echten, G., Schroder, M., Schnabel, D., and Suzuki, K. (1992) Biochem. Soc. Trans. 20, 695-699
19. Ahn, V. E., Faull, K. F., Whitelegge, J. P., Fluharty, A. L., and Prive, G. G. (2003) Proc. Natl. Acad. Sci. U. S. A. 100, 38-43
20. Qi, X., and Grabowski, G. A. (2001) J. Biol. Chem. 276, 27010-27017
21. Qi, X., and Grabowski, G. A. (1998) Biochemistry 37, 11544-11554
22. Qi, X., Leonova, T., and Grabowski, G. A. (1994) J. Biol. Chem. 269, 16746-16753
23. Harzer, K., Paton, B. C., Christomanou, H., Chatelut, M., Levade, T., Hiraiwa, M., and O'Brien, J. S. (1997) FEBS Lett. 417, 270-274
24. Azuma, N., O'Brien, J. S., Moser, H. W., and Kishimoto, Y. (1994) Arch Biochem. Biophys. 311, 354-357
25. Gatt, S., Dinur, T., Osiecki, K., Desnick, R. J., and Grabowski, G. A. (1985) Enzyme 33, 109-119
26. Chatelut, M., Harzer, K., Christomanou, H., Feunteun, J., Pieraggi, M. T., Paton, B. C., Kishimoto, Y., O'Brien, J. S., Basile, J. P., Thiers, J. C., Salvayre, R., and Levade, T. (1997) Clin. Chim. Acta 262, 61-76
27. Sun, Y., Qi, X., Witte, D., Ponce, E., Kondoh, K., Quinn, B., and Grabowski, G. (2002) Mol. Genet. Metab. 76, 271
28. Xu, Y. H., Ponce, E., Sun, Y., Leonova, T., Bove, K., Witte, D., and Grabowski, G. A. (1996) Pediatr. Res. 39, 313-322
29. Sun, Y., Witte, D. P., Jin, P., and Grabowski, G. A. (2002) Biochem. J. 370, 557-566
30. Grinde, B., and Seglen, P. O. (1980) Biochim. Biophys. Acta 632, 73-86
31. Sandhoff, K., Kolter, T., and Van Echten-Deckert, G. (1998) Ann. N. Y. Acad. Sci. 845, 139-151
32. Bergmann, J. E., and Grabowski, G. A. (1989) Am. J. Hum. Genet. 44, 741-750
33. Fujibayashi, S., and Wenger, D. A. (1986) J. Biol. Chem. 261, 15339-15343
34. Pshezhetsky, A. V., and Potier, M. (1996) J. Biol. Chem. 271, 28359-28365
35. van der Spoel, A., Bonten, E., and d'Azzo, A. (1998) EMBO J. 17, 1588-1597
36. Liu, Y., Hoffmann, A., Grinberg, A., Westphal, H., McDonald, M. P., Miller, K. M., Crawley, J. N., Sandhoff, K., Suzuki, K., and Proia, R. L. (1997) Proc. Natl. Acad. Sci. U. S. A. 94, 8138-8143
37. Sandhoff, K., Harzer, K., and Furst, W. (1995) in The Metabolic and Molecular Bases of Inherited Disease (Scriver, C. R., Beaudet, A. L., and Valle, D., eds) 7th Ed., pp. 2427-2442, McGraw-Hill, New York
38. Stevens, R. L., Fluharty, A. L., Kihara, H., Kaback, M. M., Shapiro, L. J., Marsh, B., Sandhoff, K., and Fischer, G. (1981) Am. J. Hum. Genet. 33, 900-906
39. Schlote, W., Harzer, K., Christomanou, H., Paton, B. C., Kustermann-Kuhn, B., Schmid, B., Seeger, J., Beudt, U., Schuster, I., and Langenbeck, U. (1991) Eur. J. Pediatr. 150, 584-591
40. Morimoto, S., Martin, B. M., Yamamoto, Y., Kretz, K. A., O'Brien, J. S., and Kishimoto, Y. (1989) Proc. Natl. Acad. Sci. U. S. A. 86, 3389-3393
41. Harzer, K., Paton, B. C., Poulos, A., Kustermann-Kuhn, B., Roggendorf, W., Grisar, T., and Popp, M. (1989) Eur. J. Pediatr. 149, 31-39
42. Leonova, T., and Grabowski, G. A. (2000) Mol. Genet. Metab. 70, 281-294
43. Wilkening, G., Linke, T., and Sandhoff, K. (1998) J. Biol. Chem. 273, 30271-30278
44. Vaccaro, A. M., Salvioli, R., Tatti, M., and Ciaffoni, F. (1999) Neurochem. Res. 24, 307-314
45. Grace, M. E., Newman, K. M., Scheinker, V., He, G.-S., Berg-Fussman, A., and Grabowski, G. A. (1994) J. Biol. Chem. 269, 2283-2291
46. Grace, M. E., Graves, P. N., Smith, F. I., and Grabowski, G. A. (1990) J. Biol. Chem. 265, 6827-6835