Genetic influences and neonatal lung disease

M Hallman, R Haataja - Seminars in neonatology, 2003 - Elsevier
Neonatal lung diseases may have a genetic background. The available studies mainly
concentrate on surfactant proteins (SP-A, SP-B) and respiratory distress syndrome. Specific …

[HTML][HTML] Pneumonitis and emphysema in sp-C gene targeted mice

SW Glasser, EA Detmer, M Ikegami, CL Na… - Journal of Biological …, 2003 - ASBMB
SP-C-deficient (SP-C−/−) mice developed a severe pulmonary disorder associated with
emphysema, monocytic infiltrates, epithelial cell dysplasia, and atypical accumulations of …

[HTML][HTML] Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic mice

JP Bridges, SE Wert, LM Nogee, TE Weaver - Journal of Biological …, 2003 - ASBMB
Surfactant Protein C (SP-C) is a secreted transmembrane protein that is exclusively
expressed by alveolar type II epithelial cells of the lung. SP-C associates with surfactant …

Deletion of exon 4 from human surfactant protein C results in aggresome formation and generation of a dominant negative

WJ Wang, S Mulugeta, SJ Russo… - Journal of cell …, 2003 - journals.biologists.com
Human surfactant protein C (hSP-C) is synthesized by the alveolar type 2 cell as a 197
amino acid integral membrane proprotein and proteolytically processed to a secreted 3.7 …

Idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis

JM Fellrath, RM Du Bois - Clinical and experimental medicine, 2003 - Springer
Idiopathic pulmonary fibrosis (IPF), synonymous with cryptogenic fibrosing alveolitis (CFA),
is a progressive and usually fatal disease of unknown cause characterized by sequential …

[HTML][HTML] Processing of surfactant protein C requires a type II transmembrane topology directed by juxtamembrane positively charged residues

S Mulugeta, MF Beers - Journal of Biological Chemistry, 2003 - ASBMB
Surfactant protein C (SP-C) is a lung-specific protein that is synthesized as a 21-kDa integral
membrane propeptide (pro-SP-C) and proteolytically processed to a 3.7-kDa secretory …

Idiopathic interstitial pneumonias: an update

M Pandit-Bhalla, L Diethelm, T Ovella… - Journal of thoracic …, 2003 - journals.lww.com
Idiopathic interstitial pneumonias (IIP) include a group of nonneoplastic, noninfectious lung
diseases with a diverse spectrum of pulmonary abnormalities. The term is somewhat …

Genetics of pulmonary fibrosis

RM Du Bois, I Kangesan… - Seminars in respiratory …, 2003 - thieme-connect.com
Identifying genetic determinants of pulmonary fibrosis is at an early stage of evolution. It is
now well recognized that" pulmonary fibrosis" covers a broad range of lung diseases …

Future Directions in Genetic Strategies for Understanding and Treating Idiopathic Pulmonary Fibrosis

A Heguy, R Kaplan, RG Crystal - Idiopathic Pulmonary Fibrosis, 2003 - taylorfrancis.com
Insights into the pathogenesis of idiopathic pulmonary fibrosis (IPF) and the strategies for
treatment of this disorder have evolved over the past 30 years. In the pregenetic era, prior to …