Surfactant Protein B Deficiency: Insights into Inherited Disorders of Lung Cell Metabolism

Current Problems in Pediatric and Adolescent Health Care

Volumn 27, Issue 9, 1997, Pages 329-345

  Hamvas, Aaron ^a,b  

^a WASHINGTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b ST LOUIS CHILDREN S HOSPITAL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

FC RECEPTOR; IGA RECEPTOR; LUNG SURFACTANT;

BIOSYNTHESIS; HUMAN; IMMUNOGLOBULIN A DEFICIENCY; LUNG; LUNG ALVEOLUS; LUNG ALVEOLUS PROTEINOSIS; LUNG TRANSPLANTATION; METABOLISM; NEONATAL RESPIRATORY DISTRESS SYNDROME; NEWBORN; PATHOLOGY; PRENATAL DEVELOPMENT; REVIEW; ULTRASTRUCTURE;

HUMANS; IGA DEFICIENCY; INFANT, NEWBORN; LUNG; LUNG TRANSPLANTATION; PULMONARY ALVEOLAR PROTEINOSIS; PULMONARY ALVEOLI; PULMONARY SURFACTANTS; RECEPTORS, FC; RESPIRATORY DISTRESS SYNDROME, NEWBORN;

EID: 0031241592     PISSN: 15385442     EISSN: None     Source Type: Journal    
DOI: 10.1016/s0045-9380(97)80028-x     Document Type: Article

References (122)

1. Clements JA, King JR. Composition of the surface active material. In: Crystal R, editor. The biochemical basis of pulmonary function. New York: Dekker; 1976.
2. Farrell PM, Avery ME. Hyaline membrane disease. Am Rev Respir Dis 1975;111:657-88.
3. Seeger W, Stohr G, Wolf HRD, Neuhof H. Alteration of surfactant function due to protein leakage: special interaction with fibrin monomer. J Appl Physiol 1985;58:326-38.
4. Nogee LM, deMello DE, Dehner LP, Colten HR. Pulmonary surfactant protein B deficiency in congenital pulmonary alveolar proteinosis. N Engl J Med 1993;328:406-10.
5. Hackett BP, Gitlin JD. The role of transcription factors in the development of the pulmonary epithelium. In: McDonald J. Lung growth and development. New York: Marcel Dekker; 1997. p. 55-80.
6. Reid L. The embryology of the lung. In: de Revck AVS, Porker R, editors. CIBA Foundation symposium on development of the lung. London: Churchill Livingstone; 1967.
7. Penny DP. The ultrastructure of epithelial cells of the distal lung. Int Rev Cytol 1988;111:231-69.
8. Lazzaro D, Price M, DeFelice H, DiLauro R. The transcription factor TFF-1 is expressed at the onset of thyroid and lung morphogenesis and in restricted regions of the foetal brain. Development 1991;113:1093-1104.
9. Bohinski RJ, DiLauro R, Whitsett JA. The lung-specific surfactant protein B gene promoter is a target for thyroid transcription factor 1 and hepatocyte nuclear factor 3, indicating common factors for organ-specific gene expression along the foregut axis. Mol Cell Biol 1994;14:5671-81.
10. Hackett BP, Bingle CD, Gitlin JD. Mechanisms of gene expression and cell fate determination in the developing pulmonary epithelium. Annu Rev Physiol 1996;58:51-71.
11. Wallen L, Han VKM. Spatial and temporal distribution of insulin-like growth factors I and II during development of rat lung. Am J Physiol 1994;267:L531-42.
12. Zhao Y, Young SL. Expression of transforming growth factor-p type II receptor in rat lung is regulated during development. Am J Physiol 1995;269:L419-26.
13. Maniscalco WM, Sinkin RA, Watkins RH, Campbell MH. Transforming growth factor-p1 modulates type II cell fibronectin and surfactant protein C expression. Am J Physiol 1994;267:L569-77.
14. Strandjord TP, Clark JG, Guralnick DE, Madtes DK. Immunolocalization of transforming growth factor-alpha, epidermal growth factor (EGF) and EGF receptor in normal and injured developing human lung. Pediatr Res 1995;38:851-6.
15. Avery ME, Mead J. Surface properties in relation to atelectasis and hyaline membrane disease. Am J Dis Child 1959;97:517-23.
16. Gluck L, Kulovich MV, Borer C, Keidel WN. The interpretation and significance of the lecithin/sphingomyelin ratio in amniotic fluid. Am J Obstet Gynecol 1974;120:142-55.
17. Hallman M, Feldman BH, Kirkpatrick E, Gluck L. Absence of phosphatidylglycerol (PG) in respiratory distress syndrome in the newborn. Pediatr Res 1977;11:714-20.
18. Hallman M, Gluck L. Formation of acidic phospholipids in rabbit lung during perinatal development. Pediatr Res 1980;14:1250-9.
19. Creuwels LAJM, van Golde LMG, Haagsman HP. The pulmonary surfactant system: biochemical and clinical aspects. Lung 1997;175:1-39.
20. Rooney SA, Young SL, Mendelson CR. Molecular and cellular processing of lung surfactant. FASEB J 1994;8:957-67.
21. Weaver TE, Whitsett JA. Function and regulation of pulmonary surfactant-associated proteins. Biochem J 1991;273:249-64.
22. Fisher JH, Mason R. Expression of pulmonary surfactant protein D in rat gastric mucosa. Am J Respir Cell Mol Biol 1995;12:13-8.
23. Williams MC, Hawgood S, Hamilton RL. Changes in lipid structure produced by surfactant proteins SP-A, SP-B, and SP-C. Am J Respir Cell Mol Biol 1991;5:41-50.
24. Suzuki Y, Fujita Y, Kogishi I. Reconstitution of tubular myelin from synthetic lipids and proteins associated with pig pulmonary surfactant. Am Rev Respir Dis 1989;140:75-81.
25. Sarin VK, Gupta S, Leung TK, Taylor VE, Ohning BL, Whitsett JA, Fox JL. Biophysical and biological activity of a synthetic 8.7-kDa hydrophobic pulmonary surfactant protein SP-B. Proc Natl Acad Sci U S A 1990;87:2633-7.
26. Longo ML, Bisagno AM, Zasadzinski JAN, Bruni R, Waring AJ. A function of lung surfactant protein SP-B. Science 1993;261:453-6.
27. Cochrane CG, Revak SD. Pulmonary surfactant protein B (SP-B): structure-function relationships. Science 1991;254:566-8.
28. Jobe AH. Phospholipid metabolism and turnover. In: Polin RA, Fox VVW, editors. Fetal and neonatal physiology. Philadelphia: WB Saunders; 1992, p. 986-95.
29. Batenburg JJ. Surfactant phospholipids: synthesis and storage. Am J Physiol 1992;262:L367-85.
30. Wright JR. Clearance and recycling of pulmonary surfactant. Am J Physiol 1990;259:L1-12.
31. Rooney SA, Smart DA, Weinhold PA, Feldman DA. Dexamethasone increase the activity but not the amount of choline-phosphate cytidylyltransferase in fetal rat lung. Biochim Biophys Acta 1990;1044:385-9.
32. Bleasdale JE, Thakur NR, Rader GR, Tesan M. Cytidine monophosphate-dependent synthesis of phosphatidylglycerol in permeabilized type II pneumocytes. Biochem J 1985;232:539-45.
33. Faridy EE. Effect of distention on release of surfactant in excised dog's lungs. Respir Physiol 1976;27:99-114.
34. Oyarzun MJ, Clements JA, Baritussio A. Ventilation enhances pulmonary alveolar clearance of radioactive dipalmitoyl phosphatidylcholine in liposomes. Am Rev Respir Dis 1980;121:709-21.
35. Chander A, Fisher AB. Regulation of lung surfactant secretion. Am J Physiol 1990;258:L241-53.
36. Rooney SA, Gobran LI, Griese M. Signal transduction mechanisms mediating surfactant phospholipid secretion in isolated type II cells. Prog Respir Res 1994;27:84-91.
37. Hallman M, Slivka S, Wozniak P, Sills J. Perinatal development of myoinositol uptake into lung cells: surfactant phosphatidylglycerol and phosphatidylinositol synthesis in the rabbit. Pediatr Res 1986;20:179-85.
38. Jobe AH, Ikegami M, Seidner SR, Pettenazzo A, Ruffini L. Surfactant phosphatidylcholine metabolism and surfactant function in preterm ventilated lambs. Am Rev Respir Dis 1989;139:352-9.
39. Jacobs H, Jobe A, Ikegami M, Jones S. Surfactant phosphatidylcholine source, fluxes and turnover times in 3-day-old and adult rabbits. J Biol Chem 1982;257:1805-10.
40. Whitsett JA, Nogee LM, Weaver TE, Horowitz AD. Human surfactant protein B: Structure, function, regulation, and genetic disease. Physiol Rev 1995;75:749-57.
41. Pilot-Matias TJ, Kister SE, Fox JL, Kropp K, Glaser SW, Whitsett JA. Structure and organization of the gene encoding human pulmonary surfactant proteolipid SP-B. DNA 1989;8:75-86.
42. Hawgood S. Latham D, Borchelt J, Damm D, White T, Benson B, Wright JR. Cell-specific posttranslational processing of the surfactant-associated protein SP-B. Am J Physiol 1993;264:L290-9.
43. Weaver TE, Sui L, Bogucki B, Dey C. Processing of surfactant protein B proprotein by a cathepsin D-like protease. Am J Physiol 1992;263:L95-103.
44. Lin S, Phillips KS, Wilder MR, Weaver TE. Structural requirements for intracellular transport of pulmonary surfactant protein B (SP-B). Biochim BiophysActa 1996;1312:177-85.
45. Lin S, Akinbi HT, Breslin JS, Weaver TE. Structural requirements for targeting of surfactant protein B to secretory granules in vitro and in vivo. J Biol Chem 1996;271:19689-95.
46. Akinbi HT, Breslin JS, Ikegami M, Iwamoto HS, Clark JC, Whitsett JA, Jobe AH, Weaver TE. Rescue of SP-B knockout mice with a truncated SP-B proprotein: function of the C-terminal propeptide. J Biol Chem 1997;272:9640-7.
47. Voorhout WF, Veenendaal T, Haagsman HP, Weaver TE, Whitsett JA, Van Golde LMG, Geuze HJ. Intracellular processing of pulmonary surfactant protein B in an endosomal/ lysosomal compartment. Am J Physiol 1992;263:L479-86.
48. Liley HG, White RT, Warr RG, Benson BJ, Hawgood S, Ballard PL. Regulation of messenger RNAs for the hydrophobic surfactant proteins in human lung. J Clin Invest 1989;83:1191-7.
49. Whitsett JA, Weaver TE, Clark JC, et al. Corticosteroid enhances surfactant proteolipid Phe and pVal synthesis and RNA in fetal lung. J Biol Chem 1987;262:15618-23.
50. Beers MF, Shuman H, Liley HG, Floors J, Gonzales LW, Ballard PL. Regulation and cellular localization of surfactant protein B in human fetal lung. Pediatr Res 1995;38:668-75.
51. Pryhuber GS, Hull WM, Fink I, et al. Ontogeny of surfactant proteins A and B in human amniotic fluid as indices of fetal lung maturity. Pediatr Res 1991;30:597-605.
52. Venkatesh VC, Lannuzzi DM, Ertsey R, Ballard PL. Differential corticosteroid regulation of the pulmonary hydrophobic surfactant proteins SP-B and SP-C. Am J Respir Cell Mol Biol 1993;8:222-8.
53. Ballard PL. Hormonal regulation of pulmonary surfactant. Baillieres Clin Endocrinol Metab 1989;3:723-53.
54. Guttentag SH, Schwartz M, Kondrachov D, Yue N, Planer BC, Ballard PL. Regulation and processing of surfactant protein B in pulmonary epithelial cells [abstract]. Pediatr Res 1995;37:62A.
55. Wispe JR, Clark JC, Warner BB, Fajardo D, Hull WE, Holtzman RB, Whitsett JA. Tumor necrosis factor-alpha inhibits expression of pulmonary surfactant protein. J Clin Invest 1990;86:1954-60.
56. Robertson B, Kobayashi T, Ganzuka M, Grossman G, Li W, Suzuki Y. Experimental neonatal respiratory failure induced by a monoclonal antibody to the hydrophobic surfactant-associated protein SP-B. Pediatr Res 1991;30:239-43.
57. Suzuki Y. Lung protein leakage and respiratory failure produced by a hybridoma making monoclonal antibody to the hydrophobic surfactant protein B. Int J Exp Pathol 1992;73 325-33.
58. Nogee LM, Garnier G, Singer L, Dietz HC, Murphy AM, Cutting GR, deMello DE, Colten HR. A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds. J Clin Invest 1994;93:1860-3.
59. Nogee LM, Ballard PL, Ballard RA, Beers MF, Planer BC, Polk L, deMello DE, Longmore WJ. A new mutation producing partial surfactant protein-B deficiency in a compound heterozygote. Pediatrics 1995;96:1046-52.
60. Nogee LM, Jacobstein D, Dietz HC. Allelic heterogeneity in surfactant protein-B deficiency [abstract]. Pediatr Res 1995;37:344A.
61. deMello DE, Nogee LM, Heyman S, Krous HF, Hussain M, Merritt TA, Hsueh W, Haas JE, Heidelberger K, Schumacher R, Colten HR. Molecular and phenotypic variability in the congenital alveolar proteinosis syndrome associated with inherited surfactant protein B deficiency. J Pediatr 1994;124:43-50.
62. Ballard PL, Gonzales LW, Gonzales J, Beers MF, Hamvas A. Synthesis of surfactant components in human lung deficient in surfactant protein B [abstract]. Am J Respir Crit Care Med 1996;153:A765.
63. Hamvas A, Nogee LM, deMello DE, Cole FS. Pathophysiology and treatment of surfactant protein-B deficiency. Biol Neonate 1995;67( Suppl 1 ):1831.
64. Hamvas A. Inherited surfactant protein-B deficiency. Adv Pediatr 1997;44:369-88.
65. Hamvas A, Cole FS, deMello DE, Moxley M, Whitsett JA, Colten HR, Nogee LM. Surfactant protein-B deficiency: antenatal diagnosis and prospective treatment with surfactant replacement. J Pediatr 1994;125:356-61.
66. Hamvas A, Nogee LM, Mallory GB, et al. Lung transplantation for treatment of infants with surfactant protein-B deficiency. J Pediatr 1997;130:231-9.
67. Gregory TJ, Longmore WJ, Moxley MA, Whitsett JA, Reed CR, Fowler AA 3d, Hudson LD, Maunder RJ, Crim C, Hyers TM. Surfactant chemical composition and biophysical activity in the acute respiratory distress syndrome. J Clin Invest 1992;88:1976-81.
68. deMello DE, Heyman S, Phelps DS, Hamvas A, Nogee L, Cole FS, Colten HR. Ultrastructure of lung in surfactant protein B deficiency. Am J Respir Cell Mol Biol 1994;11:230-9.
69. Clark JC, Wert SE, Bachurski CJ, et al. Targeted disruption of the surfactant protein-B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice. Proc Natl Acad Sci U S A 1995;92:7794-8.
70. Vorbroker DK, Nogee LM, Whitsett JA. Aberrant processing of surfactant protein C in hereditary SP-B deficiency. Am J Physiol 1995;268:L647-56.
71. Claypool WD, Rogers RM, Matuschak GM. Update on the clinical diagnosis, management, and pathogenesis of pulmonary alveolar proteinosis (phospholipidosis). Chest 1984;85:550-8.
72. Haworth JC, Hoogstraten J, Taylor H. Thymic alymphoplasia. Arch Dis Child 1967;42:40-54.
73. Rosen SH, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N Engl J Med 1958;258:1123-42.
74. Parto K, Kallajoki M, Aho H, Simell O. Pulmonary alveolar proteinosis and glomerulonephritis in lysinuric protein intolerance: case reports and autopsy findings of four pediatric patients. Hum Pathol 1994;25:400-7.
75. Lieschke GJ, Stanley E, Grail D, Hodgson G, Sinickas V, Gall JAM, Sinclair RA, Dunn AR. Mice lacking both microphage-and granulocyte-macrophage colony-stimulating factor have macrophages and coexistent osteopetrosis and severe lung disease. Blood 1994;84:27-35.
76. Stanley E, Lieschke GJ, Grail D, Metcalf D, Hosgeson G, Gall JAM, Maher DW, Cebon J, Sinickas V, Dunn AR. Granulocyte-macrophage colony-stimulating factor deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology. Proc Natl Acad Sci U S A 1994;91:5592-6.
77. Dranoff G, Crawford AD, Sadelain M, Ream B, Rashid A, Bronson RT, Dickersin GR, Bachurski CJ, Mark EL, Whitsett JA, Mulligan RC. Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis. Science 1994;264:713-6.
78. IL3 receptor-deficient mice are normal. Immunity 1995;2:211-22.
79. Herman TE, Nogee LM, McAlister WH, Dehner LP. Surfactant protein B deficiency: radiographic manifestations. Pediatr Radiol 1993;23:373-5.
80. Moulton SL, Krous HF. Merit TA, Odell RM, Gangitano E, Cornish JD. Congenital pulmonary alveolar proteinosis: failure of treatment with extracorporeal life support. J Pediatr 1992;120:297-302.
81. Yusen R, Cohen A, Ham vas A. Normal lung function in subjects heterozygous for SP-B deficiency. Pediatr Res 1997;41:274A.
82. Clark JC, Weaver TE, Iwamoto HS, Ikegami M, Jobe AH, Hull WM, Whitsett JA. Decreased lung compliance and air trapping in heterozygous SP-B deficient mice. Am J Respir Cell Mol Biol 1997;16:46-52.
83. Mahut B, Delacourt C, Scheinmann P, de Blic J, Mani TM, Fournet JC, Bellon G. Pulmonary alveolar proteinosis: experience with eight pediatric cases and a review. Pediatrics 1996;97:117-22.
84. Spock A. Treatment of congenital alveolar proteinosis [letter]. J Pediatr 1993;123:495.
85. Abman SH, Kinsella JP. Inhaled nitric oxide therapy of pulmonary hypertension and respiratory failure in premature and term neonates. Adv Pharmacol 1995;34:457-74.
86. Leach CL, Greenspan JS, Rubenstein SD, ShafferTH, Wolfson MR, Jackson JC, DeLemos R, Fuhrman BP. Partial liquid ventilation with perflubron in premature infants with severe respiratory distress syndrome. N Engl J Med 1996;335:761-7.
87. MacLaughlin EF, Woo MS, Horn MV, Keens TG, Starnes VA. Lung function changes following infant lung transplantation [abstract]. Am J Respir Crit Care Med 1996;153:A657.
88. Sweet SC, Spray TL, Huddleston CB, Mendeloff E, Canter CE, Balzer DT, Bridges ND, Cohen AH, Mallory GB Jr. Pediatric lung transplantation at St. Louis Children's Hospital 1990-1995. Am J Respir Crit Care Med 1997;155:1027-35.
89. King E, Shackelford G, Hamvas A. High frequency oscillation with neuromuscular blockade promotes gas exchange in SP-B deficient infants [abstract]. Am J Respir Crit Care Med 1997;155:A236.
90. Spray TL, Mallory GB, Canter CB, Huddleston CB. Pediatric lung transplantation. Indications, techniques, and early results. J Thorac Cardiovasc Surg 1994;107:990-1000.
91. Pryhuber GS, Bachurski C, Hirsch R, Bacon A, Whitsett JA. Tumor necrosis factor-alpha decreases surfactant protein B mRNA in murine lung. Am J Physiol 1996;270:L714-21.
92. Miyazaki Y, Araki K, Vesin C, Garcia I, Kapanci Y, Whitsett JA, Piguet PF, Vassalli P. Expression of a tumor necrosis factor-α transgene in murine lung causes lymphocytic and fibrosing alveolitis. J Clin Invest 1995;96:250-9.
93. Korfhagen TR, Swantz RJ, Wed SE, McCarty JM, Kerlakian CB, Glasser SW, Whitsett JA. Respiratory epithelial cell expression of human transforming growth factor-α induces lung fibrosis in transgenic mice. J Clin Invest 1994;93:1691-9.
94. Findlay RD, Taeusch HW, Walther FJ. Surfactant replacement therapy for meconium aspiration syndrome. Pediatrics 1996;97:48-52.
95. Holm BA, Notter RN. Effects of hemoglobin and cell membrane lipids on pulmonary surfactant activity. J Appl Physiol 1987;63:1434-42.
96. Kobayashi T, Nitta K, Ganzuka M, et al. Inactivation of exogenous surfactant by pulmonary edema fluid. Pediatr Res 1991;29:353-6.
97. Halliday HL, Speer CP, Robedson B. Treatment of severe meconium aspiration syndrome with porcine surfactant. Eur J Pediatr 1996;155:1047-51.
98. Nicholas TE, Doyle IR, Bersten AD. Surfactant replacement therapy in ARDS: white knight or noise in the system? Thorax 1997;52:195-7.
99. Herting E, Sun B, Jarstrand C, Curstedt T, Robertson B. Surfactant improves lung function and mitigates bacterial growth in immature ventilated rabbits with experimentally induced neonatal group B streptococcal pneumonia. Arch Dis Child 1997;76:F3-8.
100. Hamvas A, DevineT, Cole FS. Surfactant therapy failure identifies infants at risk for pulmonary mortality. Am J Dis Child 1993;147:665-8.
101. Kuint J, Reichman B, Neumann L, Shinwell ES. Prognostic value of the immediate response to surfactant. Arch Dis Child 1994;71:F170-3.
102. Collaborative Group on Antenatal Steroid Therapy. Effect of antenatal dexamethasone administration on the prevention of respiratory distress syndrome. Am J Obstet Gynecol 1981;141:276-87.
103. National Institute of Health. Effect of corticosteroids for fetal maturation on perinatal outcomes: NIH consensus statement, vol 12, number 2. Bethesda: National Institutes of Health; 1994, p. 1-24.
104. Boshier DP, Holloway H, Liggins GC, Marshall RJ. Morphometric analyses of the effects of thyrotropin-releasing hormone and cortisol on the lungs of fetal sheep. J Dev Physiol 1989;12:49-54.
105. Avery ME, Gatewood OB, Brumley G. Transient tachypnoea of the newborn: possible delayed resorption of fluid at birth. Am J Dis Child 1966;111:380-5.
106. Milner AD, Saunders RA, Hopkin IE. Effects of delivery by cesarean section on lung mechanics and lung volume in the human neonate. Arch Dis Child 1978;53:545-8.
107. Waiters DV, Oliver RE. The role of catecholamines in lung liquid absorption at birth. Pediatr Res 1978;12:239-42.
108. Strang LB. Fetal lung liquid: secretion and reabsorption. Physiol Rev 1991;71:991-1016.
109. Bland RD. Formation of fetal lung liquid and its removal near birth. In: Polin RA, Fox VVW, editors. Fetal and neonatal physiology. Philadelphia: WB Saunders; 1992, p. 782-9.
110. O'Brodovich H, Hannam V, Seear M, Mullen JB. Amiloride impairs lung liquid clearance in newborn guinea pigs. J Appl Physiol 1990;68:1758-62.
111. Hummler E, Barker P, Gatzy J, Beermann F, Verdumo C, Schmidt A, Boucher R, Rossier BC. Early death due to defective neonatal lung liquid clearance in α ENaC-deficient mice. Nat Genet 1996;12:325-8.
112. Hamvas A, Wise PH, Yang RK, Wampler NS, Noguchi A, Maurer MM, Walentik CA, Schramm WF, Cole FS. The influence of the wider use of surfactant therapy on neonatal mortality among blacks and whites. N Engl J Med 1996;334:1635-40.
113. Hulsey TC, Alexander GR, Robillard PY, Annibale DJ, Keenan A. Hyaline membrane disease: the role of ethnicity and maternal risk characteristics. Am J Obstet Gynecol 1993;168: 572-6.
114. Robillard PY, Hulsey TC, Alexander GR, Sergent MP, deCaunes F, Papiernik E. Hyaline membrane disease in black newborns: does fetal lung maturation occur earlier? Eur J Obstet Gynecol Reprod Biol 1994;55:157-61.
115. Piper JM, Langer O. Does maternal diabetes delay fetal pulmonary maturity? Am J Obstet Gynecol 1993;168:783-6.
116. Kreidberg JA, Donovan M J, Goldstein SL, Rennke H, Shepherd K, Jones RC, Jaenisch R. Alpha 3 beta 1 integrin has a crucial role in kidney and lung organogenesis. Development 1996;122:3537-47.
117. Muglia L, Jacobson L, Dikkes P, Majzoub JA. Corticotropin-releasing hormone deficiency reveals major fetal but not adult corticosteroid need. Nature 1995;373:427-32.
118. Moens CB, Auerbach AB, Conlon RA, Joyner AL, Rossant J. A targeted mutation reveals a role for N-myc in branching morphogenesis in the embryonic mouse lung. Genes Dev 1992;6:691-704.
119. Kaartinen V, Voncken JW, Shuler C, Warburton D, Bu D, Heisterkamp N, Groffen J. Abnormal lung development and cleft palate in mice lacking TGF-beta 3 indicates defects of epithelial-mesenchymal interaction. Nat Genet 1995;11:415-21.
120. Miettinen P J, Warburton D, Bu D, Zhao JS, Berger JE, Minoo P, Koivisto T, Allen L, Dobbs L, Werb Z, Derynck R. Impaired lung branching morphogenesis in the absence of functional EGF receptor. Dev Biol 1997;186:224-36.
121. Ikegami M, Korfhagen TR, Bruno MD, Whitsett JA, Jobe AH. Surfactant metabolism in surfactant protein A-deficient mice. Am J Physiol 1997;272:L479-85.
122. Stripp BR, Lund J, Mango GW, Doyen KC, Johnston C, Hultenby K, Nord M, Whitsett JA. Clara cell secretory protein: a determinant of PCB bioaccumulation in mammals. Am J Physiol 1996;271:L656-64.