Pulmonary alveolar proteinosis: progress in the first 44 years

JF Seymour, JJ Presneill - … journal of respiratory and critical care …, 2002 - atsjournals.org
Pulmonary alveolar proteinosis is a rare clinical syndrome that was first described in 1958.
Subsequently, over 240 case reports and small series have described at least 410 cases in …

Hydrophobic surfactant proteins in lung function and disease

JA Whitsett, TE Weaver - New England Journal of Medicine, 2002 - Mass Medical Soc
Pulmonary surfactants consist of phospholipids and the hydrophobic proteins surfactant
protein B and surfactant protein C. These surfactants keep the alveoli in the lung open to the …

[图书][B] Lung surfactants: basic science and clinical applications

RH Notter - 2000 - api.taylorfrancis.com
Integrating basic and clinical research on the biophysical and physiological functions of
pulmonary surfactants, this practical reference presents thorough, cutting-edge coverage on …

Diseases of pulmonary surfactant homeostasis

JA Whitsett, SE Wert, TE Weaver - Annual Review of Pathology …, 2015 - annualreviews.org
Advances in physiology and biochemistry have provided fundamental insights into the role
of pulmonary surfactant in the pathogenesis and treatment of preterm infants with respiratory …

Lysoglycerophospholipids in chronic inflammatory disorders: the PLA2/LPC and ATX/LPA axes

I Sevastou, E Kaffe, MA Mouratis, V Aidinis - Biochimica et Biophysica Acta …, 2013 - Elsevier
Lysophosphatidylcholine (LPC) and lysophosphatidic acid (LPA), the most prominent
lysoglycerophospholipids, are emerging as a novel class of inflammatory lipids, joining …

Lipid–protein and protein–protein interactions in the pulmonary surfactant system and their role in lung homeostasis

O Cañadas, B Olmeda, A Alonso… - International journal of …, 2020 - mdpi.com
Pulmonary surfactant is a lipid/protein complex synthesized by the alveolar epithelium and
secreted into the airspaces, where it coats and protects the large respiratory air–liquid …

Genetic disorders of surfactant dysfunction

SE Wert, JA Whitsett, LM Nogee - Pediatric and …, 2009 - journals.sagepub.com
Mutations in the genes encoding the surfactant proteins B and C (SP-B and SP-C) and the
phospholipid transporter, ABCA3, are associated with respiratory distress and interstitial …

Alterations in SP-B and SP-C expression in neonatal lung disease

LM Nogee - Annu. Rev. Physiol., 2004 - annualreviews.org
▪ Abstract The hydrophobic surfactant proteins, SP-B and SP-C, have important roles in
surfactant function. The importance of these proteins in normal lung function is highlighted …

Genetic disorders of the surfactant system: focus on adult disease

CHM van Moorsel, JJ van der Vis… - European Respiratory …, 2021 - Eur Respiratory Soc
Genes involved in the production of pulmonary surfactant are crucial for the development
and maintenance of healthy lungs. Germline mutations in surfactant-related genes cause a …

Interstitial lung diseases in children

A Clement, N Nathan, R Epaud, B Fauroux… - Orphanet journal of rare …, 2010 - Springer
Interstitial lung disease (ILD) in infants and children comprises a large spectrum of rare
respiratory disorders that are mostly chronic and associated with high morbidity and …