Niemann-pick C1 protein: Obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization

Proceedings of the National Academy of Sciences of the United States of America

Volumn 96, Issue 3, 1999, Pages 805-810

  Watari, Hidemichi ^a   Blanchette Mackie, E Joan ^b   Dwyer, Nancy K ^b   Glick, Jane M ^a   Patel, Shutish ^c   Neufeld, Edward B ^b   Brady, Roscoe O ^d   Pentchev, Peter G ^d   Strauss III, Jerome F ^a,e  

^a HOSPITAL OF THE UNIVERSITY OF PENNSYLVANIA   (United States)

^b NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES   (United States)

^c Vet Admin Connecticut Hlthcare S   (United States)

^d NATIONAL INSTITUTES OF HEALTH   (United States)

^e NONE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

AMINO TERMINAL SEQUENCE; ANIMAL CELL; ARTICLE; CHOLESTEROL TRANSPORT; HUMAN; LYSOSOME; MOLECULAR CLONING; NIEMANN PICK DISEASE; NONHUMAN; PRIORITY JOURNAL; PROTEIN DOMAIN; PROTEIN TARGETING; PROTEIN TRANSPORT;

AMINO ACID SUBSTITUTION; ANIMALS; CARRIER PROTEINS; CHO CELLS; CHOLESTEROL; CRICETINAE; ENDOPLASMIC RETICULUM; FILIPIN; GREEN FLUORESCENT PROTEINS; HUMANS; LUMINESCENT PROTEINS; LYSOSOMES; MEMBRANE GLYCOPROTEINS; MUTAGENESIS, SITE-DIRECTED; NIEMANN-PICK DISEASES; PROTEINS; RECOMBINANT PROTEINS; SEQUENCE DELETION; TRANSFECTION;

ANIMALIA; CRICETINAE; CRICETULUS GRISEUS;

EID: 0033514298     PISSN: 00278424     EISSN: None     Source Type: Journal    
DOI: 10.1073/pnas.96.3.805     Document Type: Article

References (29)

1. Pentchev, P. G., Vanier, M. T., Suzuki, K. & Patterson, M. C. (1995) in The Metabolic and Molecular Basis of Inherited Diseases, eds. Scriver, C. R., Beaudet, A. L., Sly, W. S. & Valle, D. D. (McGraw-Hill, New York), pp. 2625-2639.
2. Brady, R. O., Filling-Katz, M., Barton, N. W. & Pentchev, P. G. (1989) Neurol. Clin. 7, 75-88.
3. Pentchev, P. G., Kruth, H. S., Comly, M. E., Butler, J. D., Vanier, M. T., Wengel, D. A. & Patel, S. (1986) J. Biol. Chem. 261, 16775-16780.
4. Pentchev, P. G., Comly, M. E., Kruth, H. S., Vanier, M. T. Wenger, D. A., Patel, S. & Brady, R. O. (1988) Proc. Natl. Acad. Sci. USA 82, 8247-8251.
5. Liscum, L. & Faust, J. R. (1987) J. Biol. Chem. 262, 17002-17008.
6. Blanchette-Mackie, E. J., Dwyer, N. K., Amende, L. M., Kruth, H. S., Butler, J. D., Sokol, J., Comly, M. E., Vanier, M. T., August, J. T., Brady, R. O. & Pentchev, P. G. (1988) Proc. Natl. Acad. Sci. USA 85, 8022-8026.
7. Argoff, C. E., Kaneski, N. K., Brown, A., Brady, R. O. & Pentchev, P. G. (1990) Biochem. Biophys. Res. Commun. 171, 38-45.
8. Pentchev, P. G., Comly, M. E., Kruth, H. S., Patel, S., Proestel, M. & Weintroub, H. (1986) J. Biol. Chem. 261, 2772-2777.
9. Dahl, N. K., Reed, K. L., Daunais, M. A., Faust, J. R. & Liscum, L. (1992) J. Biol. Chem. 267, 4889-4896.
10. Dahl, N. K., Gutheil, W. G. & Liscum, L. (1993) J. Biol. Chem. 268, 16979-16986.
11. Cadigan, K. M., Spillane, D. M. & Chang, T. Y. (1990) J. Cell Biol. 110, 295-308.
12. Chang, T. Y., Hasan, M. T., Chin, J., Chang, C. C., Spillane, D. M. & Chen, J. (1997) Curr. Opin. Lipid 8, 65-71.
13. Carstea, E. D., Morris, J. A., Coleman, K. G., Loftus, S. K., Zhang, D., Cummings, C., Gu, J., Rosenfeld, M. A., Pavan, W. J., Krizman, D. B., et al. (1997) Science 277, 228-231.
14. Gu, J. Z., Carstea, E. D., Cummings, C., Morris, J. A., Loftus, S. K., Zhang, D., Coleman, K. G., Cooney, A. M., Comly, M. E., Fandino, L., et al. (1997) Proc. Natl. Acad. Sci. USA 94, 7378-7383.
15. Loftus, S. K., Morris, J. A., Carstea, E. D., Gu, J. Z., Cummings, C., Brown, A., Ellison, J., Ohno, K., Rosenfeld, M. A., Tahle, D. A., et al. (1997) Science 277, 232-235.
16. Letourneur, F. & Klausner, R. D. (1992) Cell 69, 1143-1157.
17. Ogata, S. & Fukuda, M. (1994) J. Biol. Chem. 269, 5210-5217.
18. Hunziker, W. & Geuze, H. J. (1996) BioEssays 18, 379-389.
19. Arakane, F., Sugawara, T., Nishino, H., Liu, Z., Holt, J. A., Pain, D., Stocco, D. M., Miller, W. L. & Strauss III, J. F. (1996) Proc. Natl. Acad. Sci. USA 93, 13731-13736.
20. Harter, C. & Mellman, I. (1992) J Cell Biol. 117, 311-324.
21. Neufeld, E. B., Wastney, M., Patel, S., Suresh, S., Cooney, A. M., Dwyer, N. K., Roff C. F., Ohno, K., Morris, J. A., Carstea, E. D., et al. (1999) J. Biol. Chem., in press.
22. Chen, J. W., Pan, W., D'Souza, M. P. & August, J. T. (1985) Arch. Biochem. Biophys. 239, 574-586.
23. Blanchette-Mackie, E. J. & Pentchev, P. (1998) in Intracellular Cholesterol Transport, eds. Freeman, D. A. & Chang, T.-Y. (Kluwer., Norwell, MA), pp. 53-73.
24. Nohturfft, A., Brown, M. S. & Goldstein, J. L. (1998) Proc. Natl. Acad. Sci. USA 95, 12848-12853.
25. Coxey, R. A., Pentchev, P. G., Campbell, G. & Blanchette-Mackie, E. J. (1993) J. Lipid Res. 34, 1165-1176.
26. Liscum, L. & Underwood, K. W. (1995) J. Biol. Chem. 276, 15443-15446.
27. Lange, Y. & Chin, J. (1997) J. Biol. Chem. 272, 17018-17022.
28. Lange, Y., Ye, J. & Steck, T. L. (1998) J. Biol. Chem. 273, 18915-18922.
29. Neufeld, E. B. (1998) in Intracellular Cholesterol Transport, eds. Freeman, D. A. & Chang, T.-Y. (Kluwer, Norwell, MA), pp. 93-107.