Pulmonary surfactant metabolism in infants lacking surfactant protein B

American Journal of Respiratory Cell and Molecular Biology

Volumn 22, Issue 3, 2000, Pages 380-391

  Beers, Michael F ^a,b,c,d   Hamvas, Aaron ^a,b,c,d   Moxley, Michael A ^a,b,c,d   Gonzales, Linda W ^a,b,c,d   Guttentag, Susan H ^a,b,c,d   Solarin, Kola O ^a,b,c,d   Longmore, William J ^a,b,c,d   Nogee, Lawrence M ^a,b,c,d   Ballard, Philip L ^a,b,c,d,e  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

^b WASHINGTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^c SAINT LOUIS UNIVERSITY   (United States)

^d JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^e CHILDREN S HOSPITAL OF PHILADELPHIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

TRITIUM;

EID: 0034129403     PISSN: 10441549     EISSN: None     Source Type: Journal    
DOI: 10.1165/ajrcmb.22.3.3645     Document Type: Article

References (57)

1. 1. Nogee, L. M., D. E. deMello, L. P. Dehner, and H. R. Colten. 1993. Brief report: deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis. N. Engl. J. Med. 328:406-410.
2. 2. deMello, D. E., L. M. Nogee, S. Heyman, H. F. Krous, M. Hussain, T. A. Merritt, W. Hsueh, J. E. Haas, K. Heidelberger, R. Schumacher, and H. R. Colten. 1994. Molecular and phenotypic variability in the congenital alveolar proteinosis syndrome associated with inherited surfactant protein B deficiency. J. Pediatr. 125:43-50.
3. 3. Nogee, L. M., G. Garnier, H. C. Dietz, L. Singer, A. M. Murphy, D. E. deMello, and H. R. Colten. 1994. A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds. J. Clin. Invest. 93:1860-1863.
4. 4. Ballard, P. L., L. M. Nogee, M. F. Beers, R. A. Ballard, B. C. Planer, L. Polk, D. E. deMello, M. A. Moxley, and W. J. Longmore. 1995. Partial deficiency of surfactant protein B in an infant with chronic lung disease. Pediatrics 96:1046-1052.
5. 5. Whitsett, J. A., L. M. Nogee, T. E. Weaver, and A. D. Horowitz. 1995. Human surfactant protein B: structure, function, regulation, and genetic disease. Physiol. Rev. 75:749-757.
6. 6. Nogee, L. M., W. M. Hull, J. A. Whitsett, and S. E. Wert. 1996. Surfactant protein (SP) B deficiency in neonates with severe respiratory failure. Pediatr. Res. 39:343A. (Abstr.)
7. 7. Pilot-Matias, T. J., S. Kister, J. L. Fox, K. Kropp. S. W. Glasser, and J. A. Whitsett. 1989. Structure and organization of the gene encoding human pulmonary surfactant proteolipid SP-B. DNA 8:75-86.
8. 8. Voorhout, W., T. Weaver, and H. Haagsman. 1993. Biosynthetic routing of pulmonary surfactant proteins in alveolar type II cells. Microsc. Res. Tech. 26:366-373.
9. 9. Guttentag, S. H., M. F. Beers, B. M. Bieler, and P. L. Ballard. 1998. Surfactant protein B processing in human fetal lung. Am. J. Physiol. 275:L559-L566.
10. 10. Eijking, E. P., D. S. Strayer, G. J. van Daal, R. Tenbrinck, T. A. Merritt, E. Hannappel, and B. Lachmann. 1991. In vivo and in vitro inactivation of bovine surfactant by an anti-surfactant monoclonal antibody. Eur. Respir. J. 4:1245-1250.
11. 11. Robertson, B., T. Kobayashi, M. Ganzuka, G. Grossmann, W. Z. Li, and Y. Suzuki. 1991. Experimental neonatal respiratory failure induced by a monoclonal antibody to the hydrophobic surfactant-associated protein SP-B. Pediatr. Res. 30:239-243.
12. 12. Mizuno, K., M. Ikegami, C. M. Chen, T. Ueda, and A. H. Jobe. 1995. Surfactant protein-B supplementation improves in vivo function of a modified natural surfactant. Pediatr. Res. 37:271-276.
13. 13. Clark, J. C., S. E. Wert, C. J. Bachurski, M. T. Stahlman, B. R. Stripp. T. E. Weaver, and J. A. Whitsett. 1995. Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice. Proc. Natl. Acad. Sci. USA 92:7794-7798.
14. 14. deMello, D. E., S. Heyman, D. S. Phelps, A. Hamvas, L. Nogee, F. S. Cole, and R. Colten. 1994. Ultrastructure of the lung in surfactant protein B deficiency. Am. J. Respir. Cell Mol. Biol. 11:230-239.
15. 15. Hamvas, A., F. S. Cole, D. E. deMello, M. Moxley, J. A. Whitsett, H. R. Colten, and L. M. Nogee. 1994. Surfactant protein B deficiency: antenatal diagnosis and prospective treatment with surfactant replacement. J. Pediatr. 125:356-361.
16. 16. Vorbroker, D. K., S. A. Profitt, L. M. Nogee, and J. A. Whitsett. 1995. Aberrant processing of surfactant protein C in hereditary SP-B deficiency. Am. J. Physiol. 268(Lung Cell. Mol. Physiol.):L647-L656.
17. 17. Ballard, P. L. 1996. Neonatal respiratory disease due to surfactant protein B deficiency. J Perinatol. 16:S28-S34.
18. 18. Hamvas, A., L. M. Nogee, G. B. Mallory, Jr., T. L. Spray, C. B. Huddleston, A. August, L. P. Dehner, D. E. deMello, M. Moxley, R. Nelson, F. S. Cole, and H. R. Colten. 1997. Lung transplantation for treatment of infants with surfactant protein B deficiency. J. Pediatr. 130:231-239.
19. 19. Ballard, P. L., L. W. Gonzales, J. Gonzales, M. F. Beers, and A. Hamvas. 1996. Synthesis of surfactant components in human lung deficient in surfactant protein B. Am. J. Respir. Crit. Care Med. 153:A765. (Abstr.)
20. 20. Wali, A., M. F. Beers, C. Dodia, S. I. Feinstein, and A. B. Fisher. 1993. ATP and adenosine 3′,5′-cyclic monophosphate stimulate the synthesis of surfactant protein A in rat lung. Am. J. Physiol. 264:L431-L437.
21. 21. Beers, M. F., H. Shuman, H. G. Liley, J. Floros, L. W. Gonzales, N. Yue, and P. L. Ballard. 1995. Surfactant protein B in human fetal lung: developmental and glucocorticoid regulation. Pediatr. Res. 38:668-675.
22. 22. Solarin, K. O., P. L. Ballard, S. H. Guttentag, C. A. Lomax, and M. F. Beers. 1997. Expression and glucocorticoid regulation of surfactant protein C in human fetal lung. Pediatr. Res. 42:356-364.
23. 23. Miller, S. A., D. D. Dykes, and H. F. Polesky. 1988. A simple salting out procedure for extracting DNA from human nucleated cells. Nucleic Acids Res. 16:1215.
24. 24. Gonzales, L. W., P. L. Ballard, R. Ertsey, and M. C. Williams. 1986. Glucocorticoids and thyroid hormones stimulate biochemical and morphological differentiation of human fetal lung in organ culture. J. Clin. Endocrinol. Metab. 62:678-691.
25. 25. White, R. T., D. Damm, J. Miller, K. Spratt, J. Schilling, S. Hawgood, B. Benson, and B. Cordell. 1985. Isolation and characterization of the human pulmonary surfactant apoprotein gene. Nature 317:361-363.
26. 26. Hawgood, S., B. Benson, and J. Schilling. 1987. Nucleotide and amino acid sequences of pulmonary surfactant protein SP-18 and evidence for cooperation between SP-18 and SP 28-36 in surfactant lipid adsorption. Proc. Natl. Acad. Sci. USA 84:66-70.
27. 27. Warr, R. G., S. Hawgood, D. I. Buckley, T. M. Crisp, J. Schilling, B. J. Benson, P. L. Ballard, J. A. Clements, and R. T. White. 1987. Low molecular weight human pulmonary surfactant protein (SP5): isolation, characterization, and cDNA and amino acid sequences. Proc. Natl Acad. Sci. USA 84: 7915-7919.
28. 28. Liley, H. G., R. T. White, R. G. Warr, B. J. Benson, S. Hawgood, and P. L. Ballard. 1989. Regulation of messenger RNAs for the hydrophobic surfactant proteins in human lung. J. Clin. Invest. 83:1191-1197.
29. 29. Ballard, P. L., R. Ertsey, L. W. Gonzales, and J. Gonzales. 1996. Transcriptional regulation of human pulmonary surfactant proteins SP-B and SP-C by glucocorticoids. Am. J. Respir. Cell Mol. Biol. 14:599-607.
30. 30. Bradford, M. M. 1976. A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding. Anal. Biochem. 72:248-254.
31. 31. Bligh, E., and W. Dyer. 1959. A rapid method of total lipid extraction and purification. Can. J. Biochem. Physiol. 37:911-917.
32. 32. Touchstone, J., J. Chen, and K. Beaver. 1980. Improved separation of phospholipids in thin layer chromatography. Lipids 15:612-617.
33. 33. Gregory, T. J., W. J. Longmore, M. A. Moxley, J. A. Whitsett, C. R. Reed, A. A. d. Fowler, L. D. Hudson, R. J. Maunder, C. Crim, and T. M. Hyers. 1991. Surfactant chemical composition and biophysical activity in acute respiratory distress syndrome. J. Clin. Invest. 88:1976-1981.
34. 34. Dittmer, J. C., and M. A. Wells. 1969. Quantitative and qualitative analysis of lipids and lipid components. Methods Enzymol. 14:482-530.
35. 35. Poorthuis, B. J., P. J. Yazaki, and K. Y. Hostetler. 1976. An improved two dimensional thin-layer chromatography system for the separation of phosphatidylglycerol and its derivatives. J. Lipid. Res. 17:433-437.
36. 36. Mason, R. J., J. Nellenbogen, and J. A. Clements. 1976. Isolation of disaturated phosphatidylcholine with osmium tetroxide. J. Lipid. Res. 17:281-284.
37. 37. Enhorning, G. 1977. Pulsating bubble technique for evaluating pulmonary surfactant. J. Appl. Physiol. 43:198-203.
38. 38. Sanders, R. L., and W. J. Longmore. 1975. Phosphatidylglycerol in rat lung: II: comparison of occurrence, composition, and metabolism in surfactant and residual lung fractions. Biochemistry 14:835-840.
39. 39. Snyder, J. M., J. M. Johnston, and C. R. Mendelson. 1981. Differentiation of type II cells of human fetal lung in vitro. Cell Tissue Res. 220:17-25.
40. 40. Witschi, H. 1991. Role of the epithelium in lung repair. Chest 99:22S-25S.
41. 41. Beers, M. F., C. Y. Kim, C. Dodia, and A. B. Fisher. 1994. Localization, synthesis, and processing of surfactant protein SP-C in rat lung analyzed by epitope-specific antipeptide antibodies. J. Biol. Chem. 269:20318-20328.
42. 42. Vorbroker, D., C. Dey, T. Weaver, and J. A. Whitsett. 1992. Surfactant protein C precursor is palmitoylated and associates with subcellular membranes. Biochim. Biophys. Acta 1105:161-169.
43. 43. Hamosh, A., B. C. Trapnell, P. L. Zeitlin, C. Montrose-Rafizadeh, B. J. Rosenstein, R. G. Crystal, and G. R. Cutting. 1991. Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis. J. Clin. Invest. 88:1880-1885.
44. 44. Eikenboom, J. C., H. K. Ploos van Amstel, P. H. Reitsma, and E. Briet. 1992. Mutations in severe, type III von Willebrand's disease in the Dutch population: candidate missense and nonsense mutations associated with reduced levels of von Willebrand factor messenger RNA. Thromb. Haemost. 68:448-454.
45. 45. Lim, S. K., C. D. Sigmund, K. W. Gross, and L. E. Maquat. 1992. Nonsense codons in human beta-globin mRNA result in the production of mRNA degradation products. Mol. Cell. Biol. 12:1149-1161.
46. 46. Longo, N., S. D. Langley, L. D. Griffin, and L. J. D. Elsas. 1992. Reduced mRNA and a nonsense mutation in the insulin-receptor gene produce heritable severe insulin resistance. Am. J. Hum. Genet. 50:998-1007.
47. 47. Mashima, Y., A. Murakami, R. G. Weleber, N. G. Kennaway, L. Clarke, T. Shiono, and G. Inana. 1992. Nonsense-codon mutations of the ornithine aminotransferase gene with decreased levels of mutant mRNA in gyrate atrophy. Am. J. Hum. Genet. 51:81-91.
48. 48. Cheng, J., and L. E. Maqual. 1993. Nonsense codons can reduce the abundance of nuclear mRNA without affecting the abundance of pre-mRNA or the half-life of cytoplasmic mRNA. Mol. Cell. Biol. 13:1892-1902.
49. 49. Zhang, S., M. J. Ruiz-Echevarria, Y. Quan, and S. W. Peltz. 1995. Identification and characterization of a sequence motif involved in nonsense-mediated mRNA decay. Mol. Cell. Biol. 15:2231-2244.
50. 50. Mustajoki, S., R. Kauppinen, P. Mustajoki, A. Suomalainen, and L. Peltonen. 1997. Steady-state transcript levels of the porphobilinogen deaminase gene in patients with acute intermittent porphyria. Genome Res. 7:1054-1060.
51. 51. Zhang, Z. X., N. Wakamatsu, E. H. Mules, G. H. Thomas, and R. A. Gravel. 1994. Impact of premature stop codons on mRNA levels in infantile Sandhoff disease. Hum. Mol. Genet. 3:139-145.
52. 52. Urlaub, G., P. J. Mitchell, C. J. Ciudad, and L. A. Chasin. 1989. Nonsense mutations in the dihydrofolate reductase gene affect RNA processing. Mol. Cell. Biol. 9:2868-2880.
53. 53. Baserga, S. J., and E. J. Benz, Jr. 1992. Beta-globin nonsense mutation: deficient accumulation of mRNA occurs despite normal cytoplasmic stability. Proc. Natl. Acad. Sci. USA 89:2935-2939.
54. 54. Peltz, S. W., A. H. Brown, and A. Jacobson. 1993. mRNA destabilization triggered by premature translational termination depends on at least three cis-acting sequence elements and one trans-acting factor. Genes Dev. 7:1737-1754.
55. 55. Hagan, K. W., M. J. Ruiz-Echevarria, Y. Quan, and S. W. Peltz. 1995. Characterization of cis-acting sequences and decay intermediates involved in nonsense-mediated mRNA turnover. Mol. Cell. Biol. 15:809-823.
56. 56. Lewis, J. F., and A. H. Jobe. 1993. Surfactant and the adult respiratory distress syndrome. Am. Rev. Respir. Dis. 147:218-233.
57. 57. Tokieda, K., J. A. Whitsett, J. C. Clark, T. E. Weaver, K. Ikeda, K. B. McConnell, A. H. Jobe, M. Ikegami, and H. S. Iwamoto. 1997. Pulmonary dysfunction in neonatal SP-B-deficienl mice. Am. J. Physiol. 273:L875-L882.