Mucopolysaccharidosis type I, unique structure of accumulated heparan sulfate and increased N-sulfotransferase activity in mice lacking α-L-iduronidase

Journal of Biological Chemistry

Volumn 286, Issue 43, 2011, Pages 37515-37524

  Holley, Rebecca J ^a   Deligny, Audrey ^b   Wei, Wei ^c   Watson, H Angharad ^a   Niñonuevo, Milady R ^c   Dagälv, Anders ^b   Leary, Julie A ^c   Bigger, Brian W ^a   Kjellén, Lena ^b   Merry, Catherine L R ^a  

^a UNIVERSITY OF MANCHESTER   (United Kingdom)

^b UPPSALA UNIVERSITY   (Sweden)

^c UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BRAIN TISSUE; COMPOSITIONAL ANALYSIS; DISEASE PROGRESSION; GLYCOSAMINOGLYCANS; HEPARAN SULFATES; IDURONIC ACID; LYSOSOMAL ENZYMES; MURINE MODEL; SECRETORY PATHWAYS; THERAPEUTIC STRATEGY;

CATALYSTS; ENZYMES; MAMMALS; TISSUE;

HYDRAULIC STRUCTURES;

HEPARAN SULFATE; LEVO IDURONIDASE; SULFOTRANSFERASE;

ANIMAL CELL; ARTICLE; BLOOD SAMPLING; BRAIN TISSUE; CONTROLLED STUDY; DISEASE COURSE; ENZYME ACTIVITY; ENZYME LOCALIZATION; ENZYME SYNTHESIS; GOLGI COMPLEX; HUMAN; HUMAN CELL; HURLER SYNDROME; INFANT; LIVER; LYSOSOME; MOUSE; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN STRUCTURE; SULFATION;

ANIMALS; DISEASE MODELS, ANIMAL; GOLGI APPARATUS; HEPARITIN SULFATE; HUMANS; IDURONIC ACID; IDURONIDASE; MICE; MICE, KNOCKOUT; MUCOPOLYSACCHARIDOSIS I; SULFOTRANSFERASES;

MURINAE; MUS;

EID: 80054802470     PISSN: 00219258     EISSN: 1083351X     Source Type: Journal    
DOI: 10.1074/jbc.M111.287474     Document Type: Article

References (42)

1. Fuller, M., Brooks, D. A., Evangelista, M., Hein, L. K., Hopwood, J. J., and Meikle, P. J. (2005) Mol. Genet. Metab. 84, 18-24
2. Braunlin, E., Mackey-Bojack, S., Panoskaltsis-Mortari, A., Berry, J. M., McElmurry, R. T., Riddle, M., Sun, L. Y., Clarke, L. A., Tolar, J., and Blazar, B. R. (2006) Pediatr. Res. 59, 27-32
3. Dusing, S. C., Rosenberg, A., Hiemenz, J. R., Piner, S., and Escolar, M. (2005) Pediatr. Phys. Ther. 17, 264-267
4. Muenzer, J., Wraith, J. E., and Clarke, L. A. (2009) Pediatrics 123, 19-29
5. Bishop, J. R., Schuksz, M., and Esko, J. D. (2007) Nature 446, 1030-1037
6. Clarke, L. A., Russell, C. S., Pownall, S., Warrington, C. L., Borowski, A., Dimmick, J. E., Toone, J., and Jirik, F. R. (1997) Hum. Mol. Genet. 6, 503-511
7. Esko, J. D., and Selleck, S. B. (2002) Annu. Rev. Biochem. 71, 435-471
8. Holmborn, K., Ledin, J., Smeds, E., Eriksson, I., Kusche-Gullberg, M., and Kjellén, L. (2004) J. Biol. Chem. 279, 42355-42358
9. Kjellén, L. (2003) Biochem. Soc. Trans. 31, 340-342
10. Kreuger, J., Jemth, P., Sanders-Lindberg, E., Eliahu, L., Ron, D., Basilico, C., Salmivirta, M., and Lindahl, U. (2005) Biochem. J. 389, 145-150
11. Pye, D. A., Vives, R. R., Turnbull, J. E., Hyde, P., and Gallagher, J. T. (1998) J. Biol. Chem. 273, 22936-22942
12. Allen, B. L., Filla, M. S., and Rapraeger, A. C. (2001) J. Cell Biol. 155, 845-858
13. Allen, B. L., and Rapraeger, A. C. (2003) J. Cell Biol. 163, 637-648
14. Forsberg, E., and Kjellén, L. (2001) J. Clin. Invest. 108, 175-180
15. Lamanna, W. C., Baldwin, R. J., Padva, M., Kalus, I., Ten Dam, G., van Kuppevelt, T. H., Gallagher, J. T., von Figura, K., Dierks, T., and Merry, C. L. (2006) Biochem. J. 400, 63-73
16. Lamanna, W. C., Kalus, I., Padva, M., Baldwin, R. J., Merry, C. L., and Dierks, T. (2007) J. Biotechnol. 129, 290-307
17. Langford-Smith, K. J., Mercer, J., Petty, J., Tylee, K., Church, H., Roberts, J., Moss, G., Jones, S., Wynn, R., Wraith, J. E., and Bigger, B. W. (2011) J. Inherit. Metab. Dis. 34, 499-508
18. Deakin, J. A., and Lyon, M. (2008) Glycobiology 18, 483-491
19. Pettersson, I., Kusche, M., Unger, E., Wlad, H., Nylund, L., Lindahl, U., and Kjellén, L. (1991) J. Biol. Chem. 266, 8044-8049
20. Pfaffl, M. W. (2001) Nucleic Acids Res. 29, e45
21. Johnson, C. E., Crawford, B. E., Stavridis, M., Ten Dam, G., Wat, A. L., Rushton, G., Ward, C. M., Wilson, V., van Kuppevelt, T. H., Esko, J. D., Smith, A., Gallagher, J. T., and Merry, C. L. (2007) Stem Cells 25, 1913-1923
22. Ledin, J., Staatz, W., Li, J. P., Götte, M., Selleck, S., Kjellén, L., and Spillmann, D. (2004) J. Biol. Chem. 279, 42732-42741
23. Maccarana, M., Sakura, Y., Tawada, A., Yoshida, K., and Lindahl, U. (1996) J. Biol. Chem. 271, 17804-17810
24. Langford-Smith, K., Arasaradnam, M., Wraith, J. E., Wynn, R., and Bigger, B. W. (2010) Mol. Genet. Metab. 99, 269-274
25. Saad, O. M., and Leary, J. A. (2004) J. Am. Soc. Mass Spectrom. 15, 1274-1286
26. Linhardt, R. J., Rice, K. G., Kim, Y. S., Lohse, D. L., Wang, H. M., and Loganathan, D. (1988) Biochem. J. 254, 781-787
27. Linker, A., and Hovingh, P. (1972) Biochemistry 11, 563-568
28. Lyon, M., Deakin, J. A., and Gallagher, J. T. (1994) J. Biol. Chem. 269, 11208-11215
29. Staples, G. O., Shi, X., and Zaia, J. (2010) J. Biol. Chem. 285, 18336-18343
30. Fux, L., Feibish, N., Cohen-Kaplan, V., Gingis-Velitski, S., Feld, S., Geffen, C., Vlodavsky, I., and Ilan, N. (2009) Cancer Res. 69, 1758-1767
31. David, G., Bai, X. M., Van der Schueren, B., Cassiman, J. J., and Van den Berghe, H. (1992) J. Cell Biol. 119, 961-975
32. Carlsson, P., Presto, J., Spillmann, D., Lindahl, U., and Kjellén, L. (2008) J. Biol. Chem. 283, 20008-20014
33. Presto, J., Thuveson, M., Carlsson, P., Busse, M., Wilén, M., Eriksson, I., Kusche-Gullberg, M., and Kjellén, L. (2008) Proc. Natl. Acad. Sci. U.S.A. 105, 4751-4756
34. Blackhall, F. H., Merry, C. L., Davies, E. J., and Jayson, G. C. (2001) Br. J. Cancer 85, 1094-1098
35. Esko, J. D., and Lindahl, U. (2001) J. Clin. Invest. 108, 169-173
36. Dhoot, G. K., Gustafsson, M. K., Ai, X., Sun, W., Standiford, D. M., and Emerson, C. P., Jr. (2001) Science 293, 1663-1666
37. Pan, C., Nelson, M. S., Reyes, M., Koodie, L., Brazil, J. J., Stephenson, E. J., Zhao, R. C., Peters, C., Selleck, S. B., Stringer, S. E., and Gupta, P. (2005) Blood 106, 1956-1964
38. Byers, S., Rozaklis, T., Brumfield, L. K., Ranieri, E., and Hopwood, J. J. (1998) Mol. Genet. Metab. 65, 282-290
39. Fuller, M., Meikle, P. J., and Hopwood, J. J. (2004) Glycobiology 14, 443-450
40. Fuller, M., Rozaklis, T., Ramsay, S. L., Hopwood, J. J., and Meikle, P. J. (2004) Pediatr. Res. 56, 733-738
41. Krivit, W., Sung, J. H., Shapiro, E. G., and Lockman, L. A. (1995) Cell Transplant. 4, 385-392
42. Wynn, R. F., Wraith, J. E., Mercer, J., O'Meara, A., Tylee, K., Thornley, M., Church, H. J., and Bigger, B. W. (2009) J. Pediatr. 154, 609-611