Molecular mechanism of α1(I)-osteogenesis imperfecta/Ehlers-Danlos syndrome: Unfolding of an N-anchor domain at the N-terminal end of the type I collagen triple helix

Journal of Biological Chemistry

Volumn 281, Issue 10, 2006, Pages 6463-6470

  Makareeva, Elena ^a   Cabral, Wayne A ^a   Marini, Joan C ^a   Leikin, Sergey ^a,b  

^a EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH AND HUMAN DEVELOPMENT   (United States)

^b NATIONAL INSTITUTES OF HEALTH   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

COLLAGEN; LIGAMENTS; MOLECULAR DYNAMICS; PROTEINS;

AMICROUNFOLDING REGION; GLYCINE; HYDROXYPROLINE RESIDUES; TYPE I COLLAGEN TRIPLE HELIX;

AMINO ACIDS;

AMINO ACID; CHYMOTRYPSIN; COLLAGEN TYPE 1; GLYCINE; HYDROXYPROLINE; PROCOLLAGEN; PROLINE; PEPTIDE FRAGMENT;

ALPHA CHAIN; AMINO TERMINAL SEQUENCE; ARTICLE; CONTROLLED STUDY; EHLERS DANLOS SYNDROME; FIBER; HUMAN; HUMAN CELL; OSTEOGENESIS IMPERFECTA; PHENOTYPE; PRIORITY JOURNAL; PROTEIN DEGRADATION; TRIPLE HELIX; AMINO ACID SEQUENCE; CHEMISTRY; CIRCULAR DICHROISM; DIFFERENTIAL SCANNING CALORIMETRY; GENETICS; METABOLISM; MOLECULAR GENETICS; POINT MUTATION; PROTEIN DENATURATION; PROTEIN FOLDING; PROTEIN TERTIARY STRUCTURE;

AMINO ACID SEQUENCE; CALORIMETRY, DIFFERENTIAL SCANNING; CIRCULAR DICHROISM; COLLAGEN TYPE I; EHLERS-DANLOS SYNDROME; HUMANS; MOLECULAR SEQUENCE DATA; OSTEOGENESIS IMPERFECTA; PEPTIDE FRAGMENTS; PHENOTYPE; POINT MUTATION; PROTEIN DENATURATION; PROTEIN FOLDING; PROTEIN STRUCTURE, TERTIARY;

EID: 33646593224     PISSN: 00219258     EISSN: 1083351X     Source Type: Journal    
DOI: 10.1074/jbc.M511830200     Document Type: Article

References (26)

1. Prockop, D. J., and Kivirikko, K. I. (1995) Annu. Rev. Biochem. 64, 403-434
2. Byers, P. H., and Cole, W. G. (2002) in Connective Tissue and Its Heritable Disorders. Molecular, Genetic, and Medical Aspects (Royce, P. M., and Steinmann, B., eds) 2nd Ed., pp. 385-430, Wiley-Liss, Inc., New York
3. Marini, J. C. (2004) in Nelson Textbook of Pediatrics (Behrman, R. E., Kliegman, R. M., and Jenson, H. B., eds) 17th Ed., pp. 2336-2338, Saunders, Philadelphia
4. Cabral, W. A., Makareeva, E., Colige, A., Letocha, A. D., Ty, J. M., Yeowell, H. N., Pals, G., Leikin, S., and Marini, J. C. (2005) J. Biol. Chem. 280, 19259-19269
5. Beighton, P., De Paepe, A., Steinmann, B., Tsipouras, P., and Wenstrup, R. J. (1998) Am. J. Med. Genet. 77, 31-37
6. Steinmann, B., Royce, P. M., and Superti-Furga, A. (2002) in Connective Tissue and Its Heritable Disorders. Molecular, Genetic, and Medical Aspects (Royce, P. M., and Steinmann, B., eds), 2nd Ed., pp. 431-523, Wiley-Liss, Inc., New York
7. Privalov, P. L. (1982) Adv. Protein Chem. 35, 1-104
8. Privalov, P. L., Tiktopulo, E. I., and Tischenko, V. M. (1979) J. Mol. Biol. 127, 203-216
9. Arnold, W. V., Fertala, A., Sieron, A. L., Hattori, H., Mechling, D., Bachinger, H. P., and Prockop, D. J. (1998) J. Biol. Chem. 273, 31822-31828
10. Cabral, W. A., Mertts, M. V., Makareeva, E., Colige, A., Tekin, M., Pandya, A., Leikin, S., and Marini, J. C. (2003) J. Biol. Chem. 278, 10006-10012
11. Leikina, E., Mertts, M. V., Kuznetsova, N., and Leikin, S. (2002) Proc. Natl. Acad. Sci. U. S. A. 99, 1314-1318
12. Davis, J. M., and Bachinger, H. P. (1993) J. Biol. Chem. 268, 25965-25972
13. Bachinger, H. P., and Davis, J. M. (1991) Int. J. Biol. Macromol. 13, 152-156
14. Persikov, A. V., Ramshaw, J. A., Kirkpatrick, A., and Brodsky, B. (2000) Biochemistry 39, 14960-14967
15. Persikov, A. V., Ramshaw, J. A., Kirkpatrick, A., and Brodsky, B. (2005) Biochemistry 44, 1414-1422
16. Persikov, A. V., Ramshaw, J. A., and Brodsky, B. (2005) J. Biol. Chem. 280, 19343-19349
17. Kuznetsova, N. V., McBride, D. J., and Leikin, S. (2003) J. Mol. Biol. 331, 191-200
18. Veis, A. (1982) Connect Tissue Res. 10, 11-24
19. Nestler, F. H., Hvidt, S., Ferry, J. D., and Veis, A. (1983) Biopolymers 22, 1747-1758
20. Sippola, M., Kaffe, S., and Prockop, D. J. (1984) J. Biol. Chem. 259, 14094-14100
21. Nicholls, A. C., Oliver, J., Renouf, D. V., Heath, D. A., and Pope, F. M. (1992) Hum. Genet. 88, 627-633
22. Dombrowski, K. E., Vogel, B. E., and Prockop, D. J. (1989) Biochemistry 28, 7107-7112
23. Raff, M. L., Craigen, W. J., Smith, L. T., Keene, D. R., and Byers, P. H. (2000) Hum. Genet. 106, 19-28
24. Eyre, D. R., Shapiro, F. D., and Aldridge, J. F. (1985) J. Biol. Chem. 260, 11322-11329
25. Holmes, D. F., Watson, R. B., Steinmann, B., and Kadler, K. E. (1993) J. Biol. Chem. 268, 15758-15765
26. Di Lullo, G. A., Sweeney, S. M., Korkko, J., Ala-Kokko, L., and San Antonio, J. D. (2002) J. Biol. Chem. 277, 4223-4231