Proceedings of the National Academy of Sciences · 1994 · 884 citations · 18 references
Life SciencesImmunologyPathologyImmunopathologyInflammationInnate ImmunityB Cell BiologyMacrophage BiologyPhagocyte BiologyPulmonary InfectionsPulmonary InflammationPulmonary PathologyAcute Lung InflammationLocal InfectionPhagocytic ActivityPulmonary Surfactant PhysiologySubclinical Infections
GM‑CSF deficiency causes lung pathology resembling human alveolar proteinosis. GM‑CSF‑deficient mice are hematologically normal but develop characteristic lung disease with surfactant accumulation, lymphocytic infiltration, and increased susceptibility to infection, indicating GM‑CSF is essential for pulmonary homeostasis.
Mice homozygous for a disrupted granulocyte/macrophage colony-stimulating factor (GM-CSF) gene develop normally and show no major perturbation of hematopoiesis up to 12 weeks of age. While most GM-CSF-deficient mice are superficially healthy and fertile, all develop abnormal lungs. There is extensive peribronchovascular infiltration with lymphocytes, predominantly B cells. Alveoli contain granular eosinophilic material and lamellar bodies, indicative of surfactant accumulation. There are numerous large intraalveolar phagocytic macrophages. Some mice have subclinical lung infections involving bacterial or fungal organisms, occasionally with focal areas of acute purulent inflammation or lobar pneumonia. Some features of this pathology resemble the human disorder alveolar proteinosis. These observations indicate that GM-CSF is not essential for the maintenance of normal levels of the major types of mature hematopoietic cells and their precursors in blood, marrow, and spleen. However, they implicate GM-CSF as essential for normal pulmonary physiology and resistance to local infection.
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Deno P. Dialynas, Zou Quan, Katherine A. Wall et al. · The Journal of Immunology · 1983 · 1.3K citations
Pulmonary Alveolar Proteinosis
Samuel H. Rosen, Benjamin Castleman, Averill A. Liebow et al. · New England Journal of Medicine · 1958 · 1.2K citations
Molecular physiology of granulocyte-macrophage colony-stimulating factor
J. D. Gasson · Blood · 1991 · 589 citations · Full text