Congenital pulmonary alveolar proteinosis is an uncommon causeof respiratory failure in full-term newborns1,2,3,4. Althoughits histopathological appearance is similar to that of the alveolarproteinosis observed in older children and adults,5 the congenitalform of the illness follows a different clinical course. Allreported infants with congenital alveolar proteinosis have diedwithin the first year of life despite maximal medical therapy.The incidence and cause of congenital alveolar proteinosis areunknown. Familial cases have been reported, and it has beenspeculated that the cause is an inborn error of surfactant metabolism4.
From the Department of Pediatrics, Division of Allergy and Pulmonary Medicine (L.M.N., H.R.C.), and the Department of Pathology (L.P.D.), Washington University School of Medicine; and the Department of Pathology, Cardinal Glennon Children's Hospital (D.E.D.) -- both in St. Louis.
Address reprint requests to Dr. Nogee at the Division of Neonatology, CMSC 210, Johns Hopkins Children's Center, 600 N. Wolfe St., Baltimore, MD 21287-3200.
References
This article has been cited by other articles:
Doan, M L, Guillerman, R P, Dishop, M K, Nogee, L M, Langston, C, Mallory, G B, Sockrider, M M, Fan, L L
(2008). Clinical, radiological and pathological features of ABCA3 mutations in children. Thorax
63: 366-373
[Abstract][Full Text]
Lhert, F., Yan, W., Biswas, S. C., Hall, S. B.
(2007). Effects of Hydrophobic Surfactant Proteins on Collapse of Pulmonary Surfactant Monolayers. Biophys. J
93: 4237-4243
[Abstract][Full Text]
Deutsch, G. H., Young, L. R., Deterding, R. R., Fan, L. L., Dell, S. D., Bean, J. A., Brody, A. S., Nogee, L. M., Trapnell, B. C., Langston, C., and the Pathology Cooperative Group:, , Albright, E. A., Askin, F. B., Baker, P., Chou, P. M., Cool, C. M., Coventry, S. C., Cutz, E., Davis, M. M., Dishop, M. K., Galambos, C., Patterson, K., Travis, W. D., Wert, S. E., White, F. V., on behalf of the ChILD Research Co-operative,
(2007). Diffuse Lung Disease in Young Children: Application of a Novel Classification Scheme. Am. J. Respir. Crit. Care Med.
176: 1120-1128
[Abstract][Full Text]
Andreeva, A. V., Kutuzov, M. A., Voyno-Yasenetskaya, T. A.
(2007). Regulation of surfactant secretion in alveolar type II cells. Am. J. Physiol. Lung Cell. Mol. Physiol.
293: L259-L271
[Abstract][Full Text]
Thomas, H., Risma, K. A., Graham, T. B., Brody, A. S., Deutsch, G. H., Young, L. R., Joseph, P. M.
(2007). A Kindred of Children With Interstitial Lung Disease. Chest
132: 221-230
[Abstract][Full Text]
Ban, N., Matsumura, Y., Sakai, H., Takanezawa, Y., Sasaki, M., Arai, H., Inagaki, N.
(2007). ABCA3 as a Lipid Transporter in Pulmonary Surfactant Biogenesis. J. Biol. Chem.
282: 9628-9634
[Abstract][Full Text]
Wang, D., Haviland, D. L., Burns, A. R., Zsigmond, E., Wetsel, R. A.
(2007). A pure population of lung alveolar epithelial type II cells derived from human embryonic stem cells. Proc. Natl. Acad. Sci. USA
104: 4449-4454
[Abstract][Full Text]
Meyer, N. J., Garcia, J. G. N.
(2007). Wading into the Genomic Pool to Unravel Acute Lung Injury Genetics. Proc Am Thorac Soc
4: 69-76
[Abstract][Full Text]
Dexter, M. E., Cosgrove, G. P., Douglas, I. S.
(2007). Managing a Rare Condition Presenting With Intractable Hypoxemic Respiratory Failure. Chest
131: 320-327
[Full Text]
Das, A., Boggaram, V.
(2007). Proteasome dysfunction inhibits surfactant protein gene expression in lung epithelial cells: mechanism of inhibition of SP-B gene expression. Am. J. Physiol. Lung Cell. Mol. Physiol.
292: L74-L84
[Abstract][Full Text]
Matsumura, Y., Ban, N., Ueda, K., Inagaki, N.
(2006). Characterization and Classification of ATP-binding Cassette Transporter ABCA3 Mutants in Fatal Surfactant Deficiency. J. Biol. Chem.
281: 34503-34514
[Abstract][Full Text]
Akei, H., Whitsett, J. A., Buroker, M., Ninomiya, T., Tatsumi, H., Weaver, T. E., Ikegami, M.
(2006). Surface tension influences cell shape and phagocytosis in alveolar macrophages. Am. J. Physiol. Lung Cell. Mol. Physiol.
291: L572-L579
[Abstract][Full Text]
Ioachimescu, O C, Kavuru, M S
(2006). Pulmonary alveolar proteinosis. Chronic Respiratory Disease
3: 149-159
[Abstract]
Sparkman, L., Chandru, H., Boggaram, V.
(2006). Ceramide decreases surfactant protein B gene expression via downregulation of TTF-1 DNA binding activity. Am. J. Physiol. Lung Cell. Mol. Physiol.
290: L351-L358
[Abstract][Full Text]
Serrano, A. G., Ryan, M., Weaver, T. E., Perez-Gil, J.
(2006). Critical Structure-Function Determinants within the N-Terminal Region of Pulmonary Surfactant Protein SP-B. Biophys. J
90: 238-249
[Abstract][Full Text]
Ikegami, M., Whitsett, J. A., Martis, P. C., Weaver, T. E.
(2005). Reversibility of lung inflammation caused by SP-B deficiency. Am. J. Physiol. Lung Cell. Mol. Physiol.
289: L962-L970
[Abstract][Full Text]
Guttentag, S. H., Akhtar, A., Tao, J.-Q., Atochina, E., Rusiniak, M. E., Swank, R. T., Bates, S. R.
(2005). Defective Surfactant Secretion in a Mouse Model of Hermansky-Pudlak Syndrome. Am. J. Respir. Cell Mol. Bio.
33: 14-21
[Abstract][Full Text]
Nesslein, L. L., Melton, K. R., Ikegami, M., Na, C.-L., Wert, S. E., Rice, W. R., Whitsett, J. A., Weaver, T. E.
(2005). Partial SP-B deficiency perturbs lung function and causes air space abnormalities. Am. J. Physiol. Lung Cell. Mol. Physiol.
288: L1154-L1161
[Abstract][Full Text]
Moya, F. R., Gadzinowski, J., Bancalari, E., Salinas, V., Kopelman, B., Bancalari, A., Kornacka, M. K., Merritt, T. A., Segal, R., Schaber, C. J., Tsai, H., Massaro, J., d'Agostino, R., for the International Surfaxin Collaborative Study,
(2005). A Multicenter, Randomized, Masked, Comparison Trial of Lucinactant, Colfosceril Palmitate, and Beractant for the Prevention of Respiratory Distress Syndrome Among Very Preterm Infants. Pediatrics
115: 1018-1029
[Abstract][Full Text]
Sinha, S. K., Lacaze-Masmonteil, T., Valls i Soler, A., Wiswell, T. E., Gadzinowski, J., Hajdu, J., Bernstein, G., Sanchez-Luna, M., Segal, R., Schaber, C. J., Massaro, J., d'Agostino, R., for the Surfaxin Therapy Against Respiratory Distr,
(2005). A Multicenter, Randomized, Controlled Trial of Lucinactant Versus Poractant Alfa Among Very Premature Infants at High Risk for Respiratory Distress Syndrome. Pediatrics
115: 1030-1038
[Abstract][Full Text]
Malcharek, S., Hinz, A., Hilterhaus, L., Galla, H.-J.
(2005). Multilayer Structures in Lipid Monolayer Films Containing Surfactant Protein C: Effects of Cholesterol and POPE. Biophys. J
88: 2638-2649
[Abstract][Full Text]
Kandasamy, S. K., Larson, R. G.
(2005). Molecular Dynamics Study of the Lung Surfactant Peptide SP-B1-25 with DPPC Monolayers: Insights into Interactions and Peptide Position and Orientation. Biophys. J
88: 1577-1592
[Abstract][Full Text]
Beatty, A. L., Malloy, J. L., Wright, J. R.
(2005). Pseudomonas aeruginosa Degrades Pulmonary Surfactant and Increases Conversion In Vitro. Am. J. Respir. Cell Mol. Bio.
32: 128-134
[Abstract][Full Text]
Latzin, P, Tredano, M, Wust, Y, de Blic, J, Nicolai, T, Bewig, B, Stanzel, F, Kohler, D, Bahuau, M, Griese, M
(2005). Anti-GM-CSF antibodies in paediatric pulmonary alveolar proteinosis. Thorax
60: 39-44
[Abstract][Full Text]
Brasch, F., Griese, M., Tredano, M., Johnen, G., Ochs, M., Rieger, C., Mulugeta, S., Muller, K.M., Bahuau, M., Beers, M.F.
(2004). Interstitial lung disease in a baby with a de novo mutation in the SFTPC gene. Eur Respir J
24: 30-39
[Abstract][Full Text]
Hamvas, A., Nogee, L. M., White, F. V., Schuler, P., Hackett, B. P., Huddleston, C. B., Mendeloff, E. N., Hsu, F.-F., Wert, S. E., Gonzales, L. W., Beers, M. F., Ballard, P. L.
(2004). Progressive Lung Disease and Surfactant Dysfunction with a Deletion in Surfactant Protein C Gene. Am. J. Respir. Cell Mol. Bio.
30: 771-776
[Abstract][Full Text]
Ueno, T., Linder, S., Na, C.-L., Rice, W. R., Johansson, J., Weaver, T. E.
(2004). Processing of Pulmonary Surfactant Protein B by Napsin and Cathepsin H. J. Biol. Chem.
279: 16178-16184
[Abstract][Full Text]
Inwald, D, Brown, K, Gensini, F, Malone, M, Goldman, A
(2004). Open lung biopsy in neonatal and paediatric patients referred for extracorporeal membrane oxygenation (ECMO). Thorax
59: 328-333
[Abstract][Full Text]
Trapnell, B. C., Whitsett, J. A., Nakata, K.
(2003). Pulmonary Alveolar Proteinosis. NEJM
349: 2527-2539
[Full Text]
Ballard, P. L., Merrill, J. D., Godinez, R. I., Godinez, M. H., Truog, W. E., Ballard, R. A.
(2003). Surfactant Protein Profile of Pulmonary Surfactant in Premature Infants. Am. J. Respir. Crit. Care Med.
168: 1123-1128
[Abstract][Full Text]
Cole, F. S.
(2003). Surfactant protein B: unambiguously necessary for adult pulmonary function. Am. J. Physiol. Lung Cell. Mol. Physiol.
285: L540-L542
[Full Text]
Melton, K. R., Nesslein, L. L., Ikegami, M., Tichelaar, J. W., Clark, J. C., Whitsett, J. A., Weaver, T. E.
(2003). SP-B deficiency causes respiratory failure in adult mice. Am. J. Physiol. Lung Cell. Mol. Physiol.
285: L543-L549
[Abstract][Full Text]
Foster, C. D., Zhang, P. X., Gonzales, L. W., Guttentag, S. H.
(2003). In Vitro Surfactant Protein B Deficiency Inhibits Lamellar Body Formation. Am. J. Respir. Cell Mol. Bio.
29: 259-266
[Abstract][Full Text]
Epaud, R., Ikegami, M., Whitsett, J. A., Jobe, A. H., Weaver, T. E., Akinbi, H. T.
(2003). Surfactant Protein B Inhibits Endotoxin-Induced Lung Inflammation. Am. J. Respir. Cell Mol. Bio.
28: 373-378
[Abstract][Full Text]
Guttentag, S., Robinson, L., Zhang, P., Brasch, F., Buhling, F., Beers, M.
(2003). Cysteine Protease Activity Is Required for Surfactant Protein B Processing and Lamellar Body Genesis. Am. J. Respir. Cell Mol. Bio.
28: 69-79
[Abstract][Full Text]
Whitsett, J. A., Weaver, T. E.
(2002). Hydrophobic Surfactant Proteins in Lung Function and Disease. NEJM
347: 2141-2148
[Full Text]
Wang, Z., Baatz, J. E., Holm, B. A., Notter, R. H.
(2002). Content-dependent activity of lung surfactant protein B in mixtures with lipids. Am. J. Physiol. Lung Cell. Mol. Physiol.
283: L897-L906
[Abstract][Full Text]
Haczku, A., Atochina, E. N., Tomer, Y., Cao, Y., Campbell, C., Scanlon, S. T., Russo, S. J., Enhorning, G., Beers, M. F.
(2002). The late asthmatic response is linked with increased surface tension and reduced surfactant protein B in mice. Am. J. Physiol. Lung Cell. Mol. Physiol.
283: L755-L765
[Abstract][Full Text]
Ikegami, M., Takabatake, N., Weaver, T. E.
(2002). Intersubunit disulfide bridge is not required for the protective role of SP-B against lung inflammation. J. Appl. Physiol.
93: 505-511
[Abstract][Full Text]
Seymour, J. F., Presneill, J. J.
(2002). Pulmonary Alveolar Proteinosis: Progress in the First 44 Years. Am. J. Respir. Crit. Care Med.
166: 215-235
[Abstract][Full Text]
Klein, J. M., McCarthy, T. A., Dagle, J. M., Snyder, J. M.
(2002). Pre- and Postnatal Lung Development, Maturation, and Plasticity: Antisense inhibition of surfactant protein A decreases tubular myelin formation in human fetal lung in vitro. Am. J. Physiol. Lung Cell. Mol. Physiol.
282: L386-L393
[Abstract][Full Text]
Strayer, M., Savani, R. C., Gonzales, L. W., Zaman, A., Cui, Z., Veszelovszky, E., Wood, E., Ho, Y.-S., Ballard, P. L.
(2002). Pre- and Postnatal Lung Development, Maturation, and Plasticity: Human surfactant protein B promoter in transgenic mice: temporal, spatial, and stimulus-responsive regulation. Am. J. Physiol. Lung Cell. Mol. Physiol.
282: L394-L404
[Abstract][Full Text]
Ikegami, M., Weaver, T. E., Conkright, J. J., Sly, P. D., Ross, G. F., Whitsett, J. A., Glasser, S. W.
(2002). Deficiency of SP-B reveals protective role of SP-C during oxygen lung injury. J. Appl. Physiol.
92: 519-526
[Abstract][Full Text]
Gupta, M, Hernandez-Juviel, J M, Waring, A J, Walther, F J
(2001). Function and inhibition sensitivity of the N-terminal segment of surfactant protein B (SP-B1-25) in preterm rabbits. Thorax
56: 871-876
[Abstract][Full Text]
Bernhard, W., Gebert, A., Vieten, G., Rau, G. A., Hohlfeld, J. M., Postle, A. D., Freihorst, J.
(2001). Pulmonary surfactant in birds: coping with surface tension in a tubular lung. Am. J. Physiol. Regul. Integr. Comp. Physiol.
281: R327-R337
[Abstract][Full Text]
Adams, C. C., Alam, M. N., Starcher, B. C., Boggaram, V.
(2001). Cell-specific and developmental regulation of rabbit surfactant protein B promoter in transgenic mice. Am. J. Physiol. Lung Cell. Mol. Physiol.
280: L724-L731
[Abstract][Full Text]
Johnson, A. L., Braidotti, P., Pietra, G. G., Russo, S. J., Kabore, A., Wang, W.-J., Beers, M. F.
(2001). Post-Translational Processing of Surfactant Protein-C Proprotein . Targeting Motifs in the NH2-Terminal Flanking Domain Are Cleaved in Late Compartments. Am. J. Respir. Cell Mol. Bio.
24: 253-263
[Abstract][Full Text]
Nogee, L. M., Dunbar, A. E., Wert, S. E., Askin, F., Hamvas, A., Whitsett, J. A.
(2001). A Mutation in the Surfactant Protein C Gene Associated with Familial Interstitial Lung Disease. NEJM
344: 573-579
[Full Text]
Paananen, R., Glumoff, V., Sormunen, R., Voorhout, W., Hallman, M.
(2001). Expression and localization of lung surfactant protein B in Eustachian tube epithelium. Am. J. Physiol. Lung Cell. Mol. Physiol.
280: L214-L220
[Abstract][Full Text]
Sherter, C. B., Mark, E. J.
(2001). Case 2-2001- A 42-Year-Old Woman with Acute Worsening of Chronic Dyspnea and Cough. NEJM
344: 212-220
[Full Text]
Guilbert, T. W., Gebb, S. A., Shannon, J. M.
(2000). Lung hypoplasia in the nitrofen model of congenital diaphragmatic hernia occurs early in development. Am. J. Physiol. Lung Cell. Mol. Physiol.
279: L1159-L1171
[Abstract][Full Text]
Haataja, R., Ramet, M., Marttila, R., Hallman, M.
(2000). Surfactant proteins A and B as interactive genetic determinants of neonatal respiratory distress syndrome. Hum Mol Genet
9: 2751-2760
[Abstract][Full Text]
Berhane, K., Margana, R. K., Boggaram, V.
(2000). Characterization of rabbit SP-B promoter region responsive to downregulation by tumor necrosis factor-alpha. Am. J. Physiol. Lung Cell. Mol. Physiol.
279: L806-L814
[Abstract][Full Text]
Pietschmann, S. M., Pison, U.
(2000). cDNA cloning of ovine pulmonary SP-A, SP-B, and SP-C: isolation of two different sequences for SP-B. Am. J. Physiol. Lung Cell. Mol. Physiol.
278: L765-L778
[Abstract][Full Text]
Beers, M. F., Hamvas, A., Moxley, M. A., Gonzales, L. W., Guttentag, S. H., Solarin, K. O., Longmore, W. J., Nogee, L. M., Ballard, P. L.
(2000). Pulmonary Surfactant Metabolism in Infants Lacking Surfactant Protein B. Am. J. Respir. Cell Mol. Bio.
22: 380-391
[Abstract][Full Text]
NOGEE, L. M., WERT, S. E., PROFFIT, S. A., HULL, W. M., WHITSETT, J. A.
(2000). Allelic Heterogeneity in Hereditary Surfactant Protein B (SP-B) Deficiency. Am. J. Respir. Crit. Care Med.
161: 973-981
[Abstract][Full Text]
Margana, R., Berhane, K., Alam, M. N., Boggaram, V.
(2000). Identification of functional TTF-1 and Sp1/Sp3 sites in the upstream promoter region of rabbit SP-B gene. Am. J. Physiol. Lung Cell. Mol. Physiol.
278: L477-L484
[Abstract][Full Text]
Cole, F. S., Hamvas, A., Rubinstein, P., King, E., Trusgnich, M., Nogee, L. M., deMello, D. E., Colten, H. R.
(2000). Population-Based Estimates of Surfactant Protein B Deficiency. Pediatrics
105: 538-541
[Abstract][Full Text]
Beck, D. C., Ikegami, M., Na, C.-L., Zaltash, S., Johansson, J., Whitsett, J. A., Weaver, T. E.
(2000). The Role of Homodimers in Surfactant Protein B Function in Vivo. J. Biol. Chem.
275: 3365-3370
[Abstract][Full Text]
CUTZ, E., WERT, S. E., NOGEE, L. M., MOORE, A. M.
(2000). Deficiency of Lamellar Bodies in Alveolar Type II Cells Associated with Fatal Respiratory Disease in a Full-term Infant. Am. J. Respir. Crit. Care Med.
161: 608-614
[Abstract][Full Text]
Naltner, A., Ghaffari, M., Whitsett, J. A., Yan, C.
(2000). Retinoic Acid Stimulation of the Human Surfactant Protein B Promoter Is Thyroid Transcription Factor 1 Site-dependent. J. Biol. Chem.
275: 56-62
[Abstract][Full Text]
Shah, P. L, Hansell, D., Lawson, P. R, Reid, K. B M, Morgan, C.
(2000). Rare diseases bullet 6: Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis. Thorax
55: 67-77
[Full Text]
Kotecha, S.
(2000). Lung growth: implications for the newborn infant. Arch. Dis. Child. Fetal Neonatal Ed.
82: 69F-74
[Full Text]
Tan, R. C., Ikegami, M., Jobe, A. H., Yao, L. Y., Possmayer, F., Ballard, P. L.
(1999). Developmental and glucocorticoid regulation of surfactant protein mRNAs in preterm lambs. Am. J. Physiol. Lung Cell. Mol. Physiol.
277: L1142-L1148
[Abstract][Full Text]
Nag, K., Munro, J. G., Inchley, K., Schurch, S., Petersen, N. O., Possmayer, F.
(1999). SP-B refining of pulmonary surfactant phospholipid films. Am. J. Physiol. Lung Cell. Mol. Physiol.
277: L1179-L1189
[Abstract][Full Text]
Huddleston, C. B., Sweet, S. C., Mallory, G. B., Hamvas, A., Mendeloff, E. N.
(1999). LUNG TRANSPLANTATION IN VERY YOUNG INFANTS. J. Thorac. Cardiovasc. Surg.
118: 796-804
[Abstract][Full Text]
Hickman-Davis, J., Matalon, S.
(1999). Surfactant Protein B Deficiency Worsens Hyperoxic Injury to the Alveolar Epithelium. Am. J. Respir. Cell Mol. Bio.
21: 449-450
[Full Text]
Tokieda, K., Iwamoto, H. S., Bachurski, C., Wert, S. E., Hull, W. M., Ikeda, K., Whitsett, J. A.
(1999). Surfactant Protein-B-Deficient Mice Are Susceptible to Hyperoxic Lung Injury. Am. J. Respir. Cell Mol. Bio.
21: 463-472
[Abstract][Full Text]
Lin, S., Na, C.-L., Akinbi, H. T., Apsley, K. S., Whitsett, J. A., Weaver, T. E.
(1999). Surfactant Protein B (SP-B) -/- Mice Are Rescued by Restoration of SP-B Expression in Alveolar Type II Cells but Not Clara Cells. J. Biol. Chem.
274: 19168-19174
[Abstract][Full Text]
Chailley-Heu, B., Chelly, N., Lelièvre-Pégorier, M., Barlier-Mur, A.-M., Merlet-Bénichou, C., Bourbon, J. R.
(1999). Mild Vitamin A Deficiency Delays Fetal Lung Maturation in the Rat. Am. J. Respir. Cell Mol. Bio.
21: 89-96
[Abstract][Full Text]
COHEN, A. H., MALLORY, G. B. Jr., ROSS, K., WHITE, D. K., MENDELOFF, E., HUDDLESTON, C. B., KEMP, J. S.
(1999). Growth of Lungs after Transplantation in Infants and in Children Younger than 3 Years of Age. Am. J. Respir. Crit. Care Med.
159: 1747-1751
[Abstract][Full Text]
Ghaffari, M., Whitsett, J. A., Yan, C.
(1999). Inhibition of hSP-B promoter in respiratory epithelial cells by a dominant negative retinoic acid receptor. Am. J. Physiol. Lung Cell. Mol. Physiol.
276: L398-L404
[Abstract][Full Text]
YUSEN, R. D., COHEN, A. H., HAMVAS, A.
(1999). Normal Lung Function in Subjects Heterozygous for Surfactant Protein-B Deficiency. Am. J. Respir. Crit. Care Med.
159: 411-414
[Abstract][Full Text]
HERMANS, C., BERNARD, A.
(1999). Lung Epithelium-specific Proteins . Characteristics and Potential Applications as Markers. Am. J. Respir. Crit. Care Med.
159: 646-678
[Full Text]
Dirksen, U., Hattenhorst, U., Schneider, P., Schroten, H., Gobel, U., Bocking, A., Muller, K.-M., Murray, R., Burdach, S.
(1998). Defective Expression of Granulocyte-Macrophage Colony-Stimulating Factor/Interleukin-3/Interleukin-5 Receptor Common beta Chain in Children With Acute Myeloid Leukemia Associated With Respiratory Failure. Blood
92: 1097-1103
[Abstract][Full Text]
Yan, C., Ghaffari, M., Whitsett, J. A., Zeng, X., Sever, Z., Lin, S.
(1998). Retinoic acid-receptor activation of SP-B gene transcription in respiratory epithelial cells. Am. J. Physiol. Lung Cell. Mol. Physiol.
275: L239-L246
[Abstract][Full Text]
Chi, X., Garnier, G., Hawgood, S., Colten, H. R.
(1998). Identification of a Novel Alternatively Spliced mRNA of Murine Pulmonary Surfactant Protein B. Am. J. Respir. Cell Mol. Bio.
19: 107-113
[Abstract][Full Text]
CLEMENTS, J. A., AVERY, M. E.
(1998). Lung Surfactant and Neonatal Respiratory Distress Syndrome. Am. J. Respir. Crit. Care Med.
157: S59-S66
[Full Text]
WHITSETT, J. A., STAHLMAN, M. T.
(1998). Impact of Advances in Physiology, Biochemistry, and Molecular Biology on Pulmonary Disease in Neonates. Am. J. Respir. Crit. Care Med.
157: S67-S71
[Full Text]
Pryhuber, G. S., Khalak, R., Zhao, Q.
(1998). Regulation of surfactant proteins A and B by TNF-alpha and phorbol ester independent of NF-kappa B. Am. J. Physiol. Lung Cell. Mol. Physiol.
274: L289-L295
[Abstract][Full Text]
Strayer, M. S., Guttentag, S. H., Ballard, P. L.
(1998). Targeting Type II and Clara Cells for Adenovirus-mediated Gene Transfer Using the Surfactant Protein B Promoter. Am. J. Respir. Cell Mol. Bio.
18: 1-9
[Abstract][Full Text]
Kobayashi, T., Tashiro, K., Yamamoto, K., Nitta, S., Ohmura, S., Suzuki, Y.
(1997). Effects of surfactant proteins SP-B and SP-C on dynamic and static mechanics of immature lungs. J. Appl. Physiol.
83: 1849-1856
[Abstract][Full Text]
Tokieda, K., Whitsett, J. A., Clark, J. C., Weaver, T. E., Ikeda, K., McConnell, K. B., Jobe, A. H., Ikegami, M., Iwamoto, H. S.
(1997). Pulmonary dysfunction in neonatal SP-B-deficient mice. Am. J. Physiol. Lung Cell. Mol. Physiol.
273: L875-L882
[Abstract][Full Text]
Akinbi, H. T., Breslin, J. S., Ikegami, M., Iwamoto, H. S., Clark, J. C., Whitsett, J. A., Jobe, A. H., Weaver, T. E.
(1997). Rescue of SP-B Knockout Mice with a Truncated SP-B Proprotein. FUNCTION OF THE C-TERMINAL PROPEPTIDE. J. Biol. Chem.
272: 9640-9647
[Abstract][Full Text]
DAVIS, A. J., JOBE, A. H., HÄFNER, D., IKEGAMI, M.
(1997). Lung Function in Premature Lambs and Rabbits Treated with a Recombinant SP-C Surfactant. Am. J. Respir. Crit. Care Med.
157: 553-559
[Abstract][Full Text]
IKEGAMI, M., WADA, K., EMERSON, G. A., REBELLO, C. M., HERNANDEZ, R. E., JOBE, A. H.
(1997). Effects of Ventilation Style on Surfactant Metabolism and Treatment Response in Preterm Lambs. Am. J. Respir. Crit. Care Med.
157: 638-644
[Abstract][Full Text]
DOYLE, I. R., DAVIDSON, K. G., BARR, H. A., NICHOLAS, T. E., PAYNE, K., PFITZNER, J.
(1997). Quantity and Structure of Surfactant Proteins Vary Among Patients with Alveolar Proteinosis. Am. J. Respir. Crit. Care Med.
157: 658-664
[Abstract][Full Text]
Margana, R. K., Boggaram, V.
(1997). Functional Analysis of Surfactant Protein B (SP-B) Promoter. Sp1, Sp3, TTF-1, and HNF-3alpha TRANSCRIPTION FACTORS ARE NECESSARY FOR LUNG CELL-SPECIFIC ACTIVATION OF SP-B GENE TRANSCRIPTION. J. Biol. Chem.
272: 3083-3090
[Abstract][Full Text]
Lin, S., Akinbi, H. T., Breslin, J. S., Weaver, T. E.
(1996). Structural Requirements for Targeting of Surfactant Protein B (SP-B) to Secretory Granules in Vitro and in Vivo. J. Biol. Chem.
271: 19689-19695
[Abstract][Full Text]
Beers, M. F., Beers, M. F.
(1996). Inhibition of Cellular Processing of Surfactant Protein C by Drugs Affecting Intracellular pH Gradients. J. Biol. Chem.
271: 14361-14370
[Abstract][Full Text]
Creuwels, L. A. J. M., Boer, E. H., Demel, R. A., Golde, L. M. G. v., Haagsman, H. P.
(1995). Neutralization of the Positive Charges of Surfactant Protein C. J. Biol. Chem.
270: 16225-16229
[Abstract][Full Text]
Colin, A. A., Mark, E. J.
(1993). Case 49-1993- A 21-Year-Old Woman with Lifelong Progressive Interstitial Lung Disease. NEJM
329: 1797-1805
[Full Text]
Glasser, S. W., Burhans, M. S., Korfhagen, T. R., Na, C.-L., Sly, P. D., Ross, G. F., Ikegami, M., Whitsett, J. A.
(2001). Altered stability of pulmonary surfactant in SP-C-deficient mice. Proc. Natl. Acad. Sci. USA
98: 6366-6371
[Abstract][Full Text]
Previous
