|
|
|
|||||||
|
|||||||||
J. Biol. Chem., Vol. 278, Issue 16, 14291-14298, April 18, 2003
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
From the SP-C-deficient (SP-C
Pneumonitis and Emphysema in sp-C Gene
Targeted Mice*
§,,,,
Division of Pulmonary Biology, Cincinnati
Children's Hospital Medical Center, Cincinnati, Ohio 45229-3039 and
the ¶ Department of Pediatrics/Division of Neonatology,
Vanderbilt University School of Medicine,
Nashville, Tennessee 37232-2370
/) mice developed a
severe pulmonary disorder associated with emphysema, monocytic
infiltrates, epithelial cell dysplasia, and atypical accumulations of
intracellular lipids in type II epithelial cells and alveolar
macrophages. Whereas alveolar and tissue surfactant phospholipid pools
were increased, levels of other surfactant proteins were not altered
(SP-B) or were modestly increased (SP-A and SP-D). Analysis of
pressure-volume curves and forced oscillatory dynamics demonstrated
abnormal respiratory mechanics typical of emphysema. Lung disease was
progressive, causing weight loss and cardiomegaly. Extensive alveolar
remodeling was accompanied by type II cell hyperplasia, obliteration of
pulmonary capillaries, and widespread expression of 
-smooth muscle
actin, indicating myofibroblast transformation in the lung parenchyma. Dysplastic epithelial cells lining conducting airways stained intensely
for the mucin, MUC5A/C. Tissue concentrations of proinflammatory cytokines were not substantially altered in the SP-C (/) mice. Production of matrix metalloproteinases (MMP-2 and MMP-9) was increased
in alveolar macrophages from SP-C (/) mice. Absence of SP-C caused
a severe progressive pulmonary disorder with histologic features
consistent with interstitial pneumonitis.
*
This work was supported by National Institutes of
Health Grants HL50046 (to S. W. G.), HL61646 (to J. A. W.,
S. W. G., M. I., and M. T. S.), HL56387 (to J. A. W. and
M. I.), HL63329, and HD11932 (to M. I.).The costs of publication of this
article were defrayed in part by the
payment of page charges. The article
must therefore be hereby marked
"advertisement" in
accordance with 18 U.S.C. Section
1734 solely to indicate this fact.
Copyright © 2003 by The American Society for Biochemistry and Molecular Biology, Inc.
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
 |
|
  K. Bein, S. C. Wesselkamper, X. Liu, M. Dietsch, N. Majumder, V. J. Concel, M. Medvedovic, M. A. Sartor, L. N. Henning, C. Venditto, et al. Surfactant-Associated Protein B Is Critical to Survival in Nickel-Induced Injury in Mice Am. J. Respir. Cell Mol. Biol., August 1, 2009; 41(2): 226 - 236. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. W. Glasser, T. L. Witt, A. P. Senft, J. E. Baatz, D. Folger, M. D. Maxfield, H. T. Akinbi, D. A. Newton, D. R. Prows, and T. R. Korfhagen Surfactant protein C-deficient mice are susceptible to respiratory syncytial virus infection Am J Physiol Lung Cell Mol Physiol, July 1, 2009; 297(1): L64 - L72. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. D. Hardie, S. W. Glasser, and J. S. Hagood Emerging Concepts in the Pathogenesis of Lung Fibrosis Am. J. Pathol., July 1, 2009; 175(1): 3 - 16. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Kotorashvili, S. J. Russo, S. Mulugeta, S. Guttentag, and M. F. Beers Anterograde Transport of Surfactant Protein C Proprotein to Distal Processing Compartments Requires PPDY-mediated Association with Nedd4 Ubiquitin Ligases J. Biol. Chem., June 12, 2009; 284(24): 16667 - 16678. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. A. Gower, S. E. Wert, and L. M. Nogee Inherited Surfactant Disorders NeoReviews, October 1, 2008; 9(10): e458 - e467. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. W. Glasser, A. P. Senft, J. A. Whitsett, M. D. Maxfield, G. F. Ross, T. R. Richardson, D. R. Prows, Y. Xu, and T. R. Korfhagen Macrophage Dysfunction and Susceptibility to Pulmonary Pseudomonas aeruginosa Infection in Surfactant Protein C-Deficient Mice J. Immunol., July 1, 2008; 181(1): 621 - 628. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Almlen, G. Stichtenoth, B. Linderholm, M. Haegerstrand-Bjorkman, B. Robertson, J. Johansson, and T. Curstedt Surfactant proteins B and C are both necessary for alveolar stability at end expiration in premature rabbits with respiratory distress syndrome J Appl Physiol, April 1, 2008; 104(4): 1101 - 1108. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. Makita, Y. Uchijima, K. Nishiyama, T. Amano, Q. Chen, T. Takeuchi, A. Mitani, T. Nagase, Y. Yatomi, H. Aburatani, et al. Multiple renal cysts, urinary concentration defects, and pulmonary emphysematous changes in mice lacking TAZ Am J Physiol Renal Physiol, March 1, 2008; 294(3): F542 - F553. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. L. Herzog, J. Van Arnam, B. Hu, J. Zhang, Q. Chen, A. M. Haberman, and D. S. Krause Lung-specific nuclear reprogramming is accompanied by heterokaryon formation and Y chromosome loss following bone marrow transplantation and secondary inflammation FASEB J, August 1, 2007; 21(10): 2592 - 2601. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. J. Greenlee, Z. Werb, and F. Kheradmand Matrix Metalloproteinases in Lung: Multiple, Multifarious, and Multifaceted Physiol Rev, January 1, 2007; 87(1): 69 - 98. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. Markart, C. Ruppert, M. Wygrecka, R. Schmidt, M. Korfei, H. Harbach, I. Theruvath, U. Pison, W. Seeger, A. Guenther, et al. Surfactant protein C mutations in sporadic forms of idiopathic interstitial pneumonias Eur. Respir. J., January 1, 2007; 29(1): 134 - 137. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. E. Lawson and J. E. Loyd The genetic approach in pulmonary fibrosis: can it provide clues to this complex disease? Proceedings of the ATS, January 1, 2006; 3(4): 345 - 349. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. Fernandez-Valdivia, Y. Zhang, S. Pai, M. L. Metzker, and A. Schumacher l7Rn6 Encodes a Novel Protein Required for Clara Cell Function in Mouse Lung Development Genetics, January 1, 2006; 172(1): 389 - 399. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Ikegami, J. A. Whitsett, P. C. Martis, and T. E. Weaver Reversibility of lung inflammation caused by SP-B deficiency Am J Physiol Lung Cell Mol Physiol, December 1, 2005; 289(6): L962 - L970. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
W. E. Lawson, V. V. Polosukhin, G. T. Stathopoulos, O. Zoia, W. Han, K. B. Lane, B. Li, E. F. Donnelly, G. E. Holburn, K. G. Lewis, et al. Increased and Prolonged Pulmonary Fibrosis in Surfactant Protein C-Deficient Mice Following Intratracheal Bleomycin Am. J. Pathol., November 1, 2005; 167(5): 1267 - 1277. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. L. Mora, C. R. Woods, A. Garcia, J. Xu, M. Rojas, S. H. Speck, J. Roman, K. L. Brigham, and A. A. Stecenko Lung infection with {gamma}-herpesvirus induces progressive pulmonary fibrosis in Th2-biased mice Am J Physiol Lung Cell Mol Physiol, November 1, 2005; 289(5): L711 - L721. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. H. Guttentag, A. Akhtar, J.-Q. Tao, E. Atochina, M. E. Rusiniak, R. T. Swank, and S. R. Bates Defective Surfactant Secretion in a Mouse Model of Hermansky-Pudlak Syndrome Am. J. Respir. Cell Mol. Biol., July 1, 2005; 33(1): 14 - 21. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
U. Lappalainen, J. A. Whitsett, S. E. Wert, J. W. Tichelaar, and K. Bry Interleukin-1{beta} Causes Pulmonary Inflammation, Emphysema, and Airway Remodeling in the Adult Murine Lung Am. J. Respir. Cell Mol. Biol., April 1, 2005; 32(4): 311 - 318. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. W. Glasser, S. K. Eszterhas, E. A. Detmer, M. D. Maxfield, and T. R. Korfhagen The murine SP-C promoter directs type II cell-specific expression in transgenic mice Am J Physiol Lung Cell Mol Physiol, April 1, 2005; 288(4): L625 - L632. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M.-C. W. Yang, J. C. Weissler, L. S. Terada, F. Deng, and Y.-S. Yang Pleiomorphic Adenoma Gene-Like-2, a Zinc Finger Protein, Transactivates the Surfactant Protein-C Promoter Am. J. Respir. Cell Mol. Biol., January 1, 2005; 32(1): 35 - 43. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W E Lawson, S W Grant, V Ambrosini, K E Womble, E P Dawson, K B Lane, C Markin, E Renzoni, P Lympany, A Q Thomas, et al. Genetic mutations in surfactant protein C are a rare cause of sporadic cases of IPF Thorax, November 1, 2004; 59(11): 977 - 980. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Griese, R. Essl, R. Schmidt, E. Rietschel, F. Ratjen, M. Ballmann, K. Paul, and for the BEAT Study Group Pulmonary Surfactant, Lung Function, and Endobronchial Inflammation in Cystic Fibrosis Am. J. Respir. Crit. Care Med., November 1, 2004; 170(9): 1000 - 1005. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Yan and H. Du Alveolus formation: what have we learned from genetic studies? J Appl Physiol, October 1, 2004; 97(4): 1543 - 1548. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. N. Atochina, J. M. Beck, A. M. Preston, A. Haczku, Y. Tomer, S. T. Scanlon, T. Fusaro, J. Casey, S. Hawgood, A. J. Gow, et al. Enhanced Lung Injury and Delayed Clearance of Pneumocystis carinii in Surfactant Protein A-Deficient Mice: Attenuation of Cytokine Responses and Reactive Oxygen-Nitrogen Species Infect. Immun., October 1, 2004; 72(10): 6002 - 6011. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. A. Whitsett, S. E. Wert, and B. C. Trapnell Genetic disorders influencing lung formation and function at birth Hum. Mol. Genet., October 1, 2004; 13(suppl_2): R207 - R215. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. A. Whitsett, C. J. Bachurski, K. C. Barnes, P. A. Bunn Jr., L. M. Case, D. N. Cook, D. Crooks, M. W. Duncan, L. Dwyer-Nield, R. C. Elston, et al. Functional Genomics of Lung Disease Am. J. Respir. Cell Mol. Biol., August 1, 2004; 31(2/S1): S1 - S81. [Full Text] [PDF]
|
|
|
|
|
|
A. Hamvas, L. M. Nogee, F. V. White, P. Schuler, B. P. Hackett, C. B. Huddleston, E. N. Mendeloff, F.-F. Hsu, S. E. Wert, L. W. Gonzales, et al. Progressive Lung Disease and Surfactant Dysfunction with a Deletion in Surfactant Protein C Gene Am. J. Respir. Cell Mol. Biol., June 1, 2004; 30(6): 771 - 776. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K.-S. Park, J. A. Whitsett, T. Di Palma, J.-H. Hong, M. B. Yaffe, and M. Zannini TAZ Interacts with TTF-1 and Regulates Expression of Surfactant Protein-C J. Biol. Chem., April 23, 2004; 279(17): 17384 - 17390. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. Buhling, N. Waldburg, A. Reisenauer, A. Heimburg, H. Golpon, and T. Welte Lysosomal cysteine proteases in the lung: role in protein processing and immunoregulation Eur. Respir. J., April 1, 2004; 23(4): 620 - 628. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
W. D. Hardie, T. D. Le Cras, K. Jiang, J. W. Tichelaar, M. Azhar, and T. R. Korfhagen Conditional expression of transforming growth factor-{alpha} in adult mouse lung causes pulmonary fibrosis Am J Physiol Lung Cell Mol Physiol, April 1, 2004; 286(4): L741 - L749. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 H. Wan, K. H. Kaestner, S.-L. Ang, M. Ikegami, F. D. Finkelman, M. T. Stahlman, P. C. Fulkerson, M. E. Rothenberg, and J. A. Whitsett Foxa2 regulates alveolarization and goblet cell hyperplasia Development, February 15, 2004; 131(4): 953 - 964. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. P. Bridges, S. E. Wert, L. M. Nogee, and T. E. Weaver Expression of a Human Surfactant Protein C Mutation Associated with Interstitial Lung Disease Disrupts Lung Development in Transgenic Mice J. Biol. Chem., December 26, 2003; 278(52): 52739 - 52746. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. L. Martin, B. Z. Moyer, M. C. Pape, B. Starcher, K. J. Leco, and R. A. W. Veldhuizen Negative impact of tissue inhibitor of metalloproteinase-3 null mutation on lung structure and function in response to sepsis Am J Physiol Lung Cell Mol Physiol, December 1, 2003; 285(6): L1222 - L1232. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. L. Ballard, J. D. Merrill, R. I. Godinez, M. H. Godinez, W. E. Truog, and R. A. Ballard Surfactant Protein Profile of Pulmonary Surfactant in Premature Infants Am. J. Respir. Crit. Care Med., November 1, 2003; 168(9): 1123 - 1128. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| All ASBMB Journals | Molecular and Cellular Proteomics |
| Journal of Lipid Research | ASBMB Today |