Interstitial lung diseases are a heterogeneous group of disordersthat are poorly understood at a molecular level.1,2 The causeis often unknown, and the histologic diagnoses used in adultsmay represent different disease processes in children.3,4,5For example, cases of desquamative interstitial pneumonitisreported in infants are often more severe and refractory totreatment than those reported in adults.6,7 Many of these casesprobably represent chronic pneumonitis of infancy.8,9 The lungsin patients with chronic pneumonitis of infancy are characterizedby interstitial thickening with mesenchymal cells, rather thanby an inflammatory infiltrate, and an alveolar infiltrate withvariable amounts of . . . [Full Text of this Article]
Case Report
Methods
Preparation and Analysis of DNA
Protein Blotting, Immunohistochemical Analysis, and Electron Microscopy
RNA Analysis
Results
Immunohistochemical Analysis
Immunoblot Analysis of Surfactant Proteins
DNA-Sequence Analysis
RNA Analysis
Discussion
Source Information
From the Division of Neonatology, Departments of Pediatrics (L.M.N., A.E.D.) and Pathology (F.A.), Johns Hopkins University School of Medicine, Baltimore; the Divisions of Neonatology and Pulmonary Biology, University of Cincinnati College of Medicine, Cincinnati (S.E.W., J.A.W.); and the Division of Newborn Medicine, Department of Pediatrics, Washington University School of Medicine, St. Louis (A.H.).
Address reprint requests to Dr. Nogee at CMSC 210, Johns Hopkins Hospital, 600 N. Wolfe St., Baltimore, MD 21287, or at lnogee@jhmi.edu.
References
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[Abstract][Full Text]
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(2008). Epithelial Endoplasmic Reticulum Stress and Apoptosis in Sporadic Idiopathic Pulmonary Fibrosis. Am. J. Respir. Crit. Care Med.
178: 838-846
[Abstract][Full Text]
Molina-Molina, M., Xaubet, A., Li, X., Abdul-Hafez, A., Friderici, K., Jernigan, K., Fu, W., Ding, Q., Pereda, J., Serrano-Mollar, A., Casanova, A., Rodriguez-Becerra, E., Morell, F., Ancochea, J., Picado, C., Uhal, B. D.
(2008). Angiotensinogen gene G-6A polymorphism influences idiopathic pulmonary fibrosis disease progression. Eur Respir J
32: 1004-1008
[Abstract][Full Text]
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(2008). Inherited Surfactant Disorders. NeoReviews
9: e458-e467
[Abstract][Full Text]
Faro, A., Hamvas, A.
(2008). Lung Transplantation for Inherited Disorders of Surfactant Metabolism. NeoReviews
9: e468-e476
[Abstract][Full Text]
Herzog, E. L., Brody, A. R., Colby, T. V., Mason, R., Williams, M. C.
(2008). Knowns and Unknowns of the Alveolus. Proc Am Thorac Soc
5: 778-782
[Abstract][Full Text]
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(2008). Usual Interstitial Pneumonia in an Adolescent With ABCA3 Mutations. Chest
134: 192-195
[Abstract][Full Text]
Glasser, S. W., Senft, A. P., Whitsett, J. A., Maxfield, M. D., Ross, G. F., Richardson, T. R., Prows, D. R., Xu, Y., Korfhagen, T. R.
(2008). Macrophage Dysfunction and Susceptibility to Pulmonary Pseudomonas aeruginosa Infection in Surfactant Protein C-Deficient Mice. J. Immunol.
181: 621-628
[Abstract][Full Text]
Behr, J., Ryu, J. H.
(2008). Pulmonary hypertension in interstitial lung disease. Eur Respir J
31: 1357-1367
[Abstract][Full Text]
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(2008). Endoplasmic reticulum stress in alveolar epithelial cells is prominent in IPF: association with altered surfactant protein processing and herpesvirus infection. Am. J. Physiol. Lung Cell. Mol. Physiol.
294: L1119-L1126
[Abstract][Full Text]
Dong, M., Bridges, J. P., Apsley, K., Xu, Y., Weaver, T. E.
(2008). ERdj4 and ERdj5 Are Required for Endoplasmic Reticulum-associated Protein Degradation of Misfolded Surfactant Protein C. Mol. Biol. Cell
19: 2620-2630
[Abstract][Full Text]
Schwartz, D. A.
(2008). Genetic Analysis of Sporadic and Familial Interstitial Pneumonia. Proc Am Thorac Soc
5: 343-347
[Abstract][Full Text]
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]
Cutz, E., Chiasson, D., Kiren, V., Barbi, E., Ventura, A., Filippone, M., Baraldi, E.
(2008). Chronic Lung Disease after Premature Birth. NEJM
358: 743-746
[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]
Mulugeta, S., Maguire, J. A., Newitt, J. L., Russo, S. J., Kotorashvili, A., Beers, M. F.
(2007). Misfolded BRICHOS SP-C mutant proteins induce apoptosis via caspase-4- and cytochrome c-related mechanisms. Am. J. Physiol. Lung Cell. Mol. Physiol.
293: L720-L729
[Abstract][Full Text]
Hunninghake, G. W., Schwarz, M. I.
(2007). State of the Art. Does Current Knowledge Explain the Pathogenesis of Idiopathic Pulmonary Fibrosis?: A Perspective. Proc Am Thorac Soc
4: 449-452
[Abstract][Full Text]
Herzog, E. L., Van Arnam, J., Hu, B., Zhang, J., Chen, Q., Haberman, A. M., Krause, D. S.
(2007). Lung-specific nuclear reprogramming is accompanied by heterokaryon formation and Y chromosome loss following bone marrow transplantation and secondary inflammation. FASEB J.
21: 2592-2601
[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]
Young, L. R., Pasula, R., Gulleman, P. M., Deutsch, G. H., McCormack, F. X.
(2007). Susceptibility of Hermansky-Pudlak Mice to Bleomycin-Induced Type II Cell Apoptosis and Fibrosis. Am. J. Respir. Cell Mol. Bio.
37: 67-74
[Abstract][Full Text]
Moya, F., Sinha, S., Gadzinowski, J., D'Agostino, R., Segal, R., Guardia, C., Mazela, J., Liu, G., on behalf of the SELECT and STAR Study Investigato,
(2007). One-Year Follow-up of Very Preterm Infants Who Received Lucinactant for Prevention of Respiratory Distress Syndrome: Results From 2 Multicenter Randomized, Controlled Trials. Pediatrics
119: e1361-e1370
[Abstract][Full Text]
Tsakiri, K. D., Cronkhite, J. T., Kuan, P. J., Xing, C., Raghu, G., Weissler, J. C., Rosenblatt, R. L., Shay, J. W., Garcia, C. K.
(2007). Adult-onset pulmonary fibrosis caused by mutations in telomerase. Proc. Natl. Acad. Sci. USA
104: 7552-7557
[Abstract][Full Text]
Tebbutt, S. J., James, A., Pare, P. D.
(2007). Single-Nucleotide Polymorphisms and Lung Disease: Clinical Implications. Chest
131: 1216-1223
[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]
Greenlee, K. J., Werb, Z., Kheradmand, F.
(2007). Matrix Metalloproteinases in Lung: Multiple, Multifarious, and Multifaceted. Physiol. Rev.
87: 69-98
[Abstract][Full Text]
Markart, P., Ruppert, C., Wygrecka, M., Schmidt, R., Korfei, M., Harbach, H., Theruvath, I., Pison, U., Seeger, W., Guenther, A., Witt, H.
(2007). Surfactant protein C mutations in sporadic forms of idiopathic interstitial pneumonias. Eur Respir J
29: 134-137
[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]
Johansson, H., Nordling, K., Weaver, T. E., Johansson, J.
(2006). The Brichos Domain-containing C-terminal Part of Pro-surfactant Protein C Binds to an Unfolded Poly-Val Transmembrane Segment. J. Biol. Chem.
281: 21032-21039
[Abstract][Full Text]
Bridges, J. P., Xu, Y., Na, C.-L., Wong, H. R., Weaver, T. E.
(2006). Adaptation and increased susceptibility to infection associated with constitutive expression of misfolded SP-C. JCB
172: 395-407
[Abstract][Full Text]
Caminati, A., Harari, S.
(2006). Smoking-related Interstitial Pneumonias and Pulmonary Langerhans Cell Histiocytosis.. Proc Am Thorac Soc
3: 299-306
[Abstract][Full Text]
Lawson, W. E., Loyd, J. E.
(2006). The genetic approach in pulmonary fibrosis: can it provide clues to this complex disease?. Proc Am Thorac Soc
3: 345-349
[Abstract][Full Text]
Lawson, W. E., Polosukhin, V. V., Stathopoulos, G. T., Zoia, O., Han, W., Lane, K. B., Li, B., Donnelly, E. F., Holburn, G. E., Lewis, K. G., Collins, R. D., Hull, W. M., Glasser, S. W., Whitsett, J. A., Blackwell, T. S.
(2005). Increased and Prolonged Pulmonary Fibrosis in Surfactant Protein C-Deficient Mice Following Intratracheal Bleomycin. Am. J. Pathol.
167: 1267-1277
[Abstract][Full Text]
Deterding, R., Fan, L. L.
(2005). Surfactant Dysfunction Mutations in Children's Interstitial Lung Disease and Beyond. Am. J. Respir. Crit. Care Med.
172: 940-941
[Full Text]
Bullard, J. E., Wert, S. E., Whitsett, J. A., Dean, M., Nogee, L. M.
(2005). ABCA3 Mutations Associated with Pediatric Interstitial Lung Disease. Am. J. Respir. Crit. Care Med.
172: 1026-1031
[Abstract][Full Text]
Mulugeta, S., Nguyen, V., Russo, S. J., Muniswamy, M., Beers, M. F.
(2005). A Surfactant Protein C Precursor Protein BRICHOS Domain Mutation Causes Endoplasmic Reticulum Stress, Proteasome Dysfunction, and Caspase 3 Activation. Am. J. Respir. Cell Mol. Bio.
32: 521-530
[Abstract][Full Text]
Grutters, J. C., du Bois, R. M.
(2005). Genetics of fibrosing lung diseases. Eur Respir J
25: 915-927
[Abstract][Full Text]
Ryu, J. H., Myers, J. L., Capizzi, S. A., Douglas, W. W., Vassallo, R., Decker, P. A.
(2005). Desquamative Interstitial Pneumonia and Respiratory Bronchiolitis-Associated Interstitial Lung Disease. Chest
127: 178-184
[Abstract][Full Text]
Yang, M.-C. W., Weissler, J. C., Terada, L. S., Deng, F., Yang, Y.-S.
(2005). Pleiomorphic Adenoma Gene-Like-2, a Zinc Finger Protein, Transactivates the Surfactant Protein-C Promoter. Am. J. Respir. Cell Mol. Bio.
32: 35-43
[Abstract][Full Text]
Percopo, S., Cameron, H.S., Nogee, L.M., Pettinato, G., Montella, S., Santamaria, F.
(2004). Variable phenotype associated with SP-C gene mutations: fatal case with the I73T mutation. Eur Respir J
24: 1072-1073
[Full Text]
Lawson, W E, Grant, S W, Ambrosini, V, Womble, K E, Dawson, E P, Lane, K B, Markin, C, Renzoni, E, Lympany, P, Thomas, A Q, Roldan, J, Scott, T A, Blackwell, T S, Phillips, J A III, Loyd, J E, du Bois, R M
(2004). Genetic mutations in surfactant protein C are a rare cause of sporadic cases of IPF. Thorax
59: 977-980
[Abstract][Full Text]
Bush, A.
(2004). Paediatric interstitial lung disease: not just kid's stuff. Eur Respir J
24: 521-523
[Full Text]
Clement, A., committee members,
(2004). Task force on chronic interstitial lung disease in immunocompetent children. Eur Respir J
24: 686-697
[Abstract][Full Text]
Whitsett, J. A., Wert, S. E., Trapnell, B. C.
(2004). Genetic disorders influencing lung formation and function at birth. Hum Mol Genet
13: R207-R215
[Abstract][Full Text]
Whitsett, J. A., Bachurski, C. J., Barnes, K. C., Bunn, P. A. Jr., Case, L. M., Cook, D. N., Crooks, D., Duncan, M. W., Dwyer-Nield, L., Elston, R. C., Fessler, M. B., Franklin, W. A., Friedman, N., Garcia, J. G. N., Geraci, M. W., Glasgow, C., Glasser, S. W., Hardie, W. D., Henning, L. M., Johnson, G. L., Kawkitinarong, K., Keith, R. L., Korfhagen, T. R., Leikauf, G. D., Liggett, S. B., Malcolm, K. C., Malkinson, A. M., Mariani, T. J., McDowell, S. A., McGraw, D. W., Medvedovic, M., Moss, J., Nogee, L. M., Nonas, S., Pacheco-Rodriguez, G., Palmer, L. J., Peters, D. G., Prows, D. R., Redline, S., Regev, A., Sartor, M. A., Schwartz, D. A., Silverman, E. K., Steagall, W. K., Stearman, R. S., Taveira-DaSilva, A., Tichelaar, J. W., Tomlinson, C. R., Tsukada, K., Weaver, T. E., Wert, S. E., Wesselkamper, S. C., Worthen, G. S., Xu, Y., Zerbe, L., Zhang, Y., Zhang, Y., Choi, A. M. K., Kaminski, N.
(2004). Functional Genomics of Lung Disease. Am. J. Respir. Cell Mol. Bio.
31: S1-S81
[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]
Park, K.-S., Whitsett, J. A., Di Palma, T., Hong, J.-H., Yaffe, M. B., Zannini, M.
(2004). TAZ Interacts with TTF-1 and Regulates Expression of Surfactant Protein-C. J. Biol. Chem.
279: 17384-17390
[Abstract][Full Text]
Buhling, F., Waldburg, N., Reisenauer, A., Heimburg, A., Golpon, H., Welte, T.
(2004). Lysosomal cysteine proteases in the lung: role in protein processing and immunoregulation. Eur Respir J
23: 620-628
[Abstract][Full Text]
Shulenin, S., Nogee, L. M., Annilo, T., Wert, S. E., Whitsett, J. A., Dean, M.
(2004). ABCA3 Gene Mutations in Newborns with Fatal Surfactant Deficiency. NEJM
350: 1296-1303
[Abstract][Full Text]
Bridges, J. P., Wert, S. E., Nogee, L. M., Weaver, T. E.
(2003). Expression of a Human Surfactant Protein C Mutation Associated with Interstitial Lung Disease Disrupts Lung Development in Transgenic Mice. J. Biol. Chem.
278: 52739-52746
[Abstract][Full Text]
Trapnell, B. C., Whitsett, J. A., Nakata, K.
(2003). Pulmonary Alveolar Proteinosis. NEJM
349: 2527-2539
[Full Text]
Mulugeta, S., Beers, M. F.
(2003). Processing of Surfactant Protein C Requires a Type II Transmembrane Topology Directed by Juxtamembrane Positively Charged Residues. J. Biol. Chem.
278: 47979-47986
[Abstract][Full Text]
Zeitlin, P. L., Gail, D. B., Banks-Schlegel, S.
(2003). Protein Processing and Degradation in Pulmonary Health and Disease. Am. J. Respir. Cell Mol. Bio.
29: 642-645
[Full Text]
Kaminski, N., Belperio, J. A., Bitterman, P. B., Chen, L., Chensue, S. W., Choi, A. M.K., Dacic, S., Dauber, J. H., du Bois, R. M., Enghild, J. J., Fattman, C. L., Grutters, J. C., Haegens, A., Hanford, L. E., Heintz, N., Henson, P. M., Hogaboam, C., Kagan, V. E., Keane, M. P., Kunkel, S. L., Land, S., Loyd, J. E., Lukacs, N., MacPherson, M., Manning, B., Manning, N., Martinelli, M., Moller, D. R., Morse, D., Mossman, B., Noble, P. W., Nowak, N., Oury, T. D., Pardo, A., Perez, A., Petty, T. L., Phan, S. H., Ramos-Nino, M. E., Ray, P., Rogers, R. M., Sato, H., Scapoli, L., Schaefer, L. M., Selman, M., Stern, M., Strollo, D. C., Tyurin, V. A., Valnickova, Z., Welsh, K. I., Witzmann, F. A., Yousem, S. A., Strieter, R. M.
(2003). Idiopathic Pulmonary Fibrosis. Am. J. Respir. Cell Mol. Bio.
29: S1-105
[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]
Glasser, S. W., Detmer, E. A., Ikegami, M., Na, C.-L., Stahlman, M. T., Whitsett, J. A.
(2003). Pneumonitis and Emphysema in sp-C Gene Targeted Mice. J. Biol. Chem.
278: 14291-14298
[Abstract][Full Text]
Wang, W.-J., Mulugeta, S., Russo, S. J., Beers, M. F.
(2003). Deletion of exon 4 from human surfactant protein C results in aggresome formation and generation of a dominant negative. J. Cell Sci.
116: 683-692
[Abstract][Full Text]
Whitsett, J. A., Weaver, T. E.
(2002). Hydrophobic Surfactant Proteins in Lung Function and Disease. NEJM
347: 2141-2148
[Full Text]
Hosia, W., Johansson, J., Griffiths, W. J.
(2002). Hydrogen/Deuterium Exchange and Aggregation of a Polyvaline and a Polyleucine {alpha}-Helix Investigated by Matrix-assisted Laser Desorption Ionization Mass Spectrometry. Mol. Cell. Proteomics
1: 592-597
[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]
Nogee, L. M.
(2002). Abnormal Expression of Surfactant Protein C and Lung Disease. Am. J. Respir. Cell Mol. Bio.
26: 641-644
[Full Text]
Brasch, F., ten Brinke, A., Johnen, G., Ochs, M., Kapp, N., Muller, K. M., Beers, M. F., Fehrenbach, H., Richter, J., Batenburg, J. J., Buhling, F.
(2002). Involvement of Cathepsin H in the Processing of the Hydrophobic Surfactant-Associated Protein C in Type II Pneumocytes. Am. J. Respir. Cell Mol. Bio.
26: 659-670
[Abstract][Full Text]
Fan, L. L., Langston, C.
(2002). Pediatric Interstitial Lung Disease: Children Are Not Small Adults. Am. J. Respir. Crit. Care Med.
165: 1466-1467
[Full Text]
Canakis, A.-M., Cutz, E., Manson, D., O'Brodovich, H.
(2002). Pulmonary Interstitial Glycogenosis: A New Variant of Neonatal Interstitial Lung Disease. Am. J. Respir. Crit. Care Med.
165: 1557-1565
[Abstract][Full Text]
Wang, W.-J., Russo, S. J., Mulugeta, S., Beers, M. F.
(2002). Biosynthesis of Surfactant Protein C (SP-C). SORTING OF SP-C PROPROTEIN INVOLVES HOMOMERIC ASSOCIATION VIA A SIGNAL ANCHOR DOMAIN. J. Biol. Chem.
277: 19929-19937
[Abstract][Full Text]
Whitsett, J. A.
(2002). Genetic Basis of Familial Interstitial Lung Disease: Misfolding or Function of Surfactant Protein C?. Am. J. Respir. Crit. Care Med.
165: 1201-1202
[Full Text]
Thomas, A. Q., Lane, K., Phillips, J. III, Prince, M., Markin, C., Speer, M., Schwartz, D. A., Gaddipati, R., Marney, A., Johnson, J., Roberts, R., Haines, J., Stahlman, M., Loyd, J. E.
(2002). Heterozygosity for a Surfactant Protein C Gene Mutation Associated with Usual Interstitial Pneumonitis and Cellular Nonspecific Interstitial Pneumonitis in One Kindred. Am. J. Respir. Crit. Care Med.
165: 1322-1328
[Abstract][Full Text]
Nogee, L. M., Dunbar, A. E. III, Wert, S., Askin, F., Hamvas, A., Whitsett, J. A.
(2002). Mutations in the Surfactant Protein C Gene Associated With Interstitial Lung Disease. Chest
121: 20S-21S
[Abstract][Full Text]
du Bois, R. M.
(2002). The Genetic Predisposition to Interstitial Lung Disease : Functional Relevance. Chest
121: 14S-20S
[Abstract][Full Text]
Conkright, J. J., Na, C.-L., Weaver, T. E.
(2002). Overexpression of Surfactant Protein-C Mature Peptide Causes Neonatal Lethality in Transgenic Mice. Am. J. Respir. Cell Mol. Bio.
26: 85-90
[Abstract][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]