Lung polymers in Z α1-antitrypsin deficiency-related emphysema

American Journal of Respiratory Cell and Molecular Biology

Volumn 18, Issue 5, 1998, Pages 670-674

  Elliott, Peter R ^a   Bilton, Diana ^a   Lomas, David A ^a  

^a UNIVERSITY OF CAMBRIDGE   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA 1 ANTITRYPSIN; POLYMER;

ALPHA 1 ANTITRYPSIN DEFICIENCY; ARTICLE; CHEMISTRY; ELECTROPHORESIS; EMPHYSEMA; HUMAN; LUNG LAVAGE; METABOLISM; METHODOLOGY; PROTEIN CONFORMATION; PROTEIN SECONDARY STRUCTURE;

ALPHA 1-ANTITRYPSIN; ALPHA 1-ANTITRYPSIN DEFICIENCY; BRONCHOALVEOLAR LAVAGE FLUID; ELECTROPHORESIS; EMPHYSEMA; HUMANS; POLYMERS; PROTEIN CONFORMATION; PROTEIN STRUCTURE, SECONDARY;

EID: 0032065494     PISSN: 10441549     EISSN: None     Source Type: Journal    
DOI: 10.1165/ajrcmb.18.5.3065     Document Type: Article

References (26)

1. Brantly, M., T. Nukiwa, and R. G. Crystal. 1988. Molecular basis of alpha-1-antitrypsin deficiency. Am. J. Med. 84(Suppl. 6A):13-31.
2. Sharp, H. L., R. A. Bridges, W. Krivit, and E. F. Freier. 1969. Cirrhosis associated with alpha-1-antitrypsin deficiency: a previously unrecognised inherited disorder. J. Lab. Clin. Med. 73:934-939.
3. 1-antitrypsin deficiency detected by screening of 200,000 infants. N. Engl. J. Med. 294:1316-1321.
4. 1-antitrypsin deficiency. N. Engl. J. Med. 314:736-739.
5. 1-antitrypsin for structure and function of scrpins. Biochemistry 28:8951-8966.
6. Stein, P. E., and R. W. Carrell. 1995. What do dysfunctional serpins tell us about molecular mobility and disease? Nature Structural Biol. 2:96-113.
7. 1-antitrypsin causes a protein folding defect. Nature Structural Biol. 2: 363-367.
8. 1-antitrypsin. Biochemistrt 32:500-508.
9. 1-antitrypsin accumulation in the liver. Nature 357:605-607.
10. 1-antitrypsin deficiency. Q. J. Med. 89:807-812.
11. 1-antitrypsin. Nature Structural Biol. 3:676-681.
12. 1-antitrypsin at 2.7Å. Structure 4:1181-1192.
13. 1-antitrypsin deficiency. Hepatology 22:514-517.
14. 53→Phe); further evidence for intracellular loopsheet polymerisation. J. Biol. Chem. 268:15333-15335.
15. 52Phe deleted) forms loop-sheet polymers in vivo: evidence for the C sheet mechanism of polymerisation. J. Biol. Chem. 270:16864-16870.
16. 1-antitrypsin. Nature Structural Biol. 3:910-911.
17. 1-antitrypsin. J. Biol. Chem. 270:5282-5288.
18. Goldenberg, D. P. 1989. Analysis of protein conformation by gel electrophoresis. In Protein Structure: A Practical Approach. T. E. Creighton, editor. IRL Press, Oxford. 225-250.
19. 1-proteinase inhibitor probed by limited proteolysis. Biochemistry 31:2720-2728.
20. 1-untitrypsin (358Met→Arg)-trypsin, and antitrypsin-elastase. Proteins: Structure, Function and Genetics 26:288-303.
21. Lawrence, D. A. 1997. The serpin-proteinase complex revealed. Nature Structural Biol. 4:339-341.
22. 1-antitrypsin contains a conformationally inactive, latent component. Eur. Respir. J. 10:672-675.
23. 1-proteinase inhibitor probed by intrinsic tryptophan fluorescence spectroscopy. Protein Sci. 5:2226-2235.
24. 1-antitrypsin by a peptide sequentially similar to β-strand s4A. Eur. J. Biochem. 194:51-56.
25. Beatty, K., J. Bieth, and J. Travis. 1980. Kinetics of association of serine proteinases with native and oxidized α-1-proteinase inhibitor and α-1-antichymotrypsin. J. Biol. Chem. 255:3931-3934.
26. 1-proteinase inhibitor: crystal structure analysis of two crystal modifications, molecular model and preliminary analysis of the implications for function. J. Mol. Biol. 177:531-556.