Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
MGD |
Granulocyte/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology.
|
8202532 |
1994 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
The underlying molecular mechanisms causing deficiencies in GM-CSF signaling are as follows: 1) high levels of neutralizing GM-CSF autoantibodies observed in autoimmune PAP; 2) mutations in CSF2RA, the gene encoding the alpha chain of the GM-CSF receptor, observed in hereditary PAP; and 3) reduced numbers and function of alveolar macrophages as a result of other clinical diseases seen in secondary PAP.
|
20623372 |
2010 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
LHGDN |
Elevated IL-10 inhibits GM-CSF synthesis in pulmonary alveolar proteinosis.
|
14567558 |
2003 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Deficiency of the granulocyte-macrophage colony-stimulating factor (GM-CSF)/interleukin-3 (IL-3)/IL-5 receptors common beta chain (betac) is a cause of fatal respiratory failure. betac deficiency manifests as pulmonary alveolar proteinosis (PAP).
|
9694696 |
1998 |
Pulmonary Alveolar Proteinosis
|
0.300 |
AlteredExpression
|
disease |
BEFREE |
Elevated gelatinase activity in pulmonary alveolar proteinosis: role of macrophage-colony stimulating factor.
|
16275889 |
2006 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
MGD |
Granulocyte-macrophage colony-stimulating factor-deficient mice have impaired resistance to blood-stage malaria.
|
11119498 |
2001 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Most cases of PAP are associated with the formation of autoantibodies against the cytokine granulocyte/macrophage colony-stimulating factor (GM-CSF), which is required for normal surfactant homeostasis and lung function.
|
19015311 |
2008 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
These results suggest that ABCG1 deficiency in PAP and GM-CSF KO alveolar macrophages is attributable to the absence of a GM-CSF-mediated PPARgamma pathway.
|
17848583 |
2007 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
We discuss the classification of PAP, the current diagnostic practice and the supplementary role of genetic testing and granulocyte-macrophage colony-stimulating factor (GM-CSF) signalling in the diagnosis of congenital and hereditary PAP.
|
31365379 |
2019 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Evidence is required regarding treatment with exogenous anti-granulocyte/macrophage colony-stimulating factor.Here, we present a 13-year-old male patient with hereditary PAP and a 15-year-old female patient with autoimmune PAP who presented with complaints of easy fatigability and weakness to emphasize the importance of keeping in mind PAP as a differential diagnosis in patients with respiratory failure findings.
|
30113436 |
2018 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease in which the abnormalities in alveolar surfactant accumulation are caused by impairments of GM-CSF pathway attributing to defects in a variety of genes.
|
28233860 |
2017 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Disseminated Cryptococcosis Due to Anti-Granulocyte-Macrophage Colony-Stimulating Factor Autoantibodies in the Absence of Pulmonary Alveolar Proteinosis.
|
28013480 |
2017 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Primary pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by accumulation of surfactant in the lungs that is presumed to be mediated by disruption of granulocyte/macrophage colony-stimulating factor (GM-CSF) signaling based on studies in genetically modified mice.
|
18955570 |
2008 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Disruption of granulocyte/macrophage colony-stimulating factor (GM-CSF) signalling causes pulmonary alveolar proteinosis (PAP).
|
21075760 |
2011 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Pulmonary alveolar proteinosis (PAP) is a syndrome of reduced GM-CSF-dependent, macrophage-mediated surfactant clearance, dysfunctional foamy alveolar macrophages, alveolar surfactant accumulation, and hypoxemic respiratory failure for which the pathogenetic mechanism is unknown.
|
30087322 |
2018 |
Pulmonary Alveolar Proteinosis
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
This study aimed to investigate the clinical efficacy of GM-CSF inhalation combined with WLL in Chinese patients with PAP.
|
31513019 |
2019 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
We have shown that activin A, a cytokine implicated in regulating B-cell proliferation, is severely deficient in alveolar macrophages from patients with pulmonary alveolar proteinosis (PAP), an autoimmune disorder characterized by surfactant accumulation and neutralizing autoantibodies to granulocyte-macrophage colony stimulating factor.
|
18803071 |
2009 |
Pulmonary Alveolar Proteinosis
|
0.300 |
GeneticVariation
|
disease |
BEFREE |
In fact, blockade of GM-CSF-initiated signaling or deletion of the PPARγ-encoding gene <i>PPARG</i> leads to functionally defective A-MØ and the onset of pulmonary alveolar proteinosis.
|
29434585 |
2018 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Because the phenotypic and immunologic abnormalities of PAP in mouse models can be corrected by GM-CSF reconstituting therapies, early clinical trials are underway utilizing administration of GM-CSF to potentially treat human PAP.
|
19666756 |
2009 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Neutralization and clearance of GM-CSF by autoantibodies in pulmonary alveolar proteinosis.
|
26077231 |
2015 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Using static and dynamic lipidomics techniques, we compared (1) tissue PC compositions and contents, and (2) in lungs, the absolute rates of synthesis in both control mice and the granulocyte-macrophage colony-stimulating factor knockout model of PAP.
|
28489415 |
2017 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
MGD |
Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis.
|
8171324 |
1994 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Understanding the mechanism of action for neutralizing antibodies that target GM-CSF is important for improving their specificity and affinity as therapeutics and, conversely, in devising strategies to reduce the effects of GM-CSF auto-antibodies in PAP.
|
29969365 |
2018 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Elevated IL-10 inhibits GM-CSF synthesis in pulmonary alveolar proteinosis.
|
14567558 |
2003 |
Pulmonary Alveolar Proteinosis
|
0.300 |
Biomarker
|
disease |
BEFREE |
Moreover, the expression of SLC7A7 is regulated by GM-CSF in monocytes, pointing to a role of y+LAT1 in the pathogenesis of LPI associated PAP.
|
21110863 |
2010 |