Rapid CommunicationPulmonary Alveolar Proteinosis Is a Disease of Decreased Availability of GM-CSF Rather than an Intrinsic Cellular Defect
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Cited by (67)
Pulmonary surfactant metabolism in the alveolar airspace: Biogenesis, extracellular conversions, recycling
2017, Annals of AnatomyCitation Excerpt :Finally, beyond disorders in surfactant production, secretion or reuptake by type II pneumocytes, an impairment of surfactant clearance and catabolism by macrophages may also cause several forms of PAP. These may be due to GMCSF mutations or autoantibodies, or to a decrease in the number of alveolar macrophages or their function, secondary to other disorders (Kitamura et al., 1999; Thomassen et al., 2000; Carey and Trapnell, 2010). It is also important to consider the implication of the lipid transporters ABCG1 and ABCA1 in the alveolar accumulation of lipids and cholesterol at PAP patients (Malur et al., 2011; Gowdy and Fessler, 2013).
Alveolar macrophage activation in obese patients with obstructive sleep apnea
2012, SurgeryCitation Excerpt :Channels included nasal pressure transducers, snore channel, recordings of abdominal and chest movement using respiratory and inductive plethysmography, pulse oximetry, and heart rate monitoring (Easy III ambulatory PSG system, Cadwell). Alveolar macrophages were derived from BAL obtained by fiberoptic bronchoscopy performed for research as described previously.17 Briefly, bronchoscopy was performed in an ambulatory setting using conscious sedation.
Lentivirus-ABCG1 instillation reduces lipid accumulation and improves lung compliance in GM-CSF knock-out mice
2011, Biochemical and Biophysical Research CommunicationsCitation Excerpt :In mice homozygous for a disrupted GM-CSF gene or for the common β-chain of the GM-CSF/IL-3/IL-5 receptor gene, an excess of pulmonary surfactant occurs which histologically resembles the human disease, pulmonary alveolar proteinosis (PAP) [18,31,36]. PAP however is characterized by the presence of neutralizing anti-GM-CSF autoantibodies that effectively block GM-CSF signaling pathways in surfactant homeostasis [22,38]. The result is that in both species, alveolar macrophages accumulate excess intracellular neutral lipids which are detectable by Oil Red O staining. [20,37].
Targeted PPARγ deficiency in alveolar macrophages disrupts surfactant catabolism
2010, Journal of Lipid ResearchAutosomal dominant and sporadic monocytopenia with susceptibility to mycobacteria, fungi, papillomaviruses, and myelodysplasia
2010, BloodCitation Excerpt :Most patients had severe or disseminated human papillomavirus (HPV) infection, whereas several also had disseminated histoplasmosis, invasive aspergillosis, or cryptococcal meningitis. Pulmonary alveolar proteinosis (PAP), a condition resulting from abnormalities in pulmonary alveolar macrophage metabolism of granulocyte-macrophage colony-stimulating factor (GM-CSF) or surfactant,10-14 developed in 5 patients with long-standing disease. All affected persons demonstrated persistent and profound peripheral monocytopenia, B-cell and NK-cell lymphocytopenia, with variable T-cell lymphocytopenia.
Molecular Basis of Pulmonary Disease
2009, Molecular Pathology: The Molecular Basis of Human Disease