Pulmonary alveolar proteinosis

BC Trapnell, JA Whitsett, K Nakata - New England Journal of …, 2003 - Mass Medical Soc
In acquired pulmonary alveolar proteinosis, lipids and proteins accumulate within the alveoli
because alveolar macrophages cannot catabolize surfactants. Surprisingly, alveolar …

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 …

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 …

Surfactant protein A and B genetic variants predispose to idiopathic pulmonary fibrosis

M Selman, HM Lin, M Montaño, AL Jenkins, A Estrada… - Human genetics, 2003 - Springer
Derangement in pulmonary surfactant or its components and alveolar collapse are common
findings in idiopathic pulmonary fibrosis (IPF). Surfactant proteins play important roles in …

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 …

Pulmonary fibrosis in families

JE Loyd - American Journal of Respiratory Cell and Molecular …, 2003 - search.proquest.com
'l9l~ amino acid proprotein with alternative splicing). Sur—factant protein C precursor protein
is an integral membrane protein that is anchored in the membrane by the hydropho—bic …

[PDF][PDF] Regulation of lung surfactant protein gene expression

V Boggaram - Front Biosci, 2003 - article.imrpress.com
Introduction 3. Cell/tissue-specific expression of surfactant protein genes 4. Developmental
regulation of surfactant protein gene expression 5. Multifactorial regulation of surfactant …

[图书][B] Neonatal respiratory disorders

A Greenough - 2003 - books.google.com
Lung disease is a major indication for the admittance of the neonate to a specialist intensive
care unit, and is a particularly common complication in the pre-term baby where the lungs …

Analysis of 40 sporadic or familial neonatal and pediatric cases with severe unexplained respiratory distress: Relationship to SFTPB

M Tredano, M Griese, J de Blic, T Lorant… - American Journal of …, 2003 - Wiley Online Library
We have analyzed surfactant protein B (SP‐B) and its encoding gene (SFTPB, MIM 178640)
in 40 unrelated pediatric patients with unexplained respiratory distress (URD). There was …