Mutations of Cystic Fibrosis Transmembrane Conductance Regulator Gene Cause a Monocyte-Selective Adhesion Deficiency.
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Summary
Data highlight the critical regulatory role of CFTR in integrin activation by chemoattractants in monocytes and identify CF as a new, cell type-selective leukocyte adhesion deficiency disease, providing new insights into CF pathogenesis.
- Type
- article
- Published
- 2016-05-13
- Cited by
- 63
- References
- 29
- OpenAlex
- https://openalex.org/W2207982651
- Semantic Scholar
- https://api.semanticscholar.org/CorpusID:207701079
Keywords
Chemotaxis, Integrin, Cystic fibrosis transmembrane conductance regulator, Immunology, Cystic fibrosis
References
- Monocytes Are Potent Facilitators of Alveolar Neutrophil Emigration During Lung Inflammation: Role of the CCL2-CCR2 Axis1
- New horizons in the treatment of cystic fibrosis
- A mouse model for the cystic fibrosis delta F508 mutation.
- Blood monocyte migration to acute lung inflammation involves both CD11/CD18 and very late activation antigen-4-dependent and independent pathways.
- Evidence for direct CFTR inhibition by CFTR(inh)-172 based on Arg347 mutagenesis.
- Cystic fibrosis transmembrane conductance regulator does not affect neutrophil migration across cystic fibrosis airway epithelial monolayers.
- Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy.
- Leukocyte adhesion deficiencies
- CFTR: a hub for kinases and crosstalk of cAMP and Ca2+
- The molecular basis of leukocyte recruitment and its deficiencies.
- Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770
- In vivo two-photon imaging reveals monocyte-dependent neutrophil extravasation during pulmonary inflammation
- Optimal complement-mediated phagocytosis of Pseudomonas aeruginosa by monocytes is cystic fibrosis transmembrane conductance regulator-dependent.
- Getting to the site of inflammation: the leukocyte adhesion cascade updated
- Chemokines and the Signaling Modules Regulating Integrin Affinity
- Regulation of conformer-specific activation of the integrin LFA-1 by a chemokine-triggered Rho signaling module
- Defective CFTR Expression and Function Are Detectable in Blood Monocytes: Development of a New Blood Test for Cystic Fibrosis
- Lower Airway Inflammation in Infants with Cystic Fibrosis Detected by Newborn Screening
- Early pulmonary inflammation in infants with cystic fibrosis.
- Bronchial mucous glands in the newborn with cystic fibrosis
Cited by
- Leukocyte Adhesion Deficiency IV. Monocyte Integrin Activation Deficiency in Cystic Fibrosis.
- CF Lung Immunity: The Role of the Macrophage
- In vivo monitoring of lung inflammation in CFTR-deficient mice
- Cystic fibrosis: a clinical view
- The impact of impaired macrophage functions in cystic fibrosis disease progression.
- Symposium Summaries
- Impaired defenses of neonatal mouse alveolar macrophage with cftr deletion are modulated by glutathione and TGFβ1
- CFTR‐dependent chloride efflux in cystic fibrosis mononuclear cells is increased by ivacaftor therapy
- How neutrophils resist shear stress at blood vessel walls: molecular mechanisms, subcellular structures, and cell–cell interactions
- Ezrin links CFTR to TLR4 signaling to orchestrate anti-bacterial immune response in macrophages
- Monocytes from patients with Primary Ciliary Dyskinesia show enhanced inflammatory properties and produce higher levels of pro-inflammatory cytokines
- Chemoattractants and cytokines in primary ciliary dyskinesia and cystic fibrosis: key players in chronic respiratory diseases
- Testing CFTR repair in cystic fibrosis patients carrying nonsense and channel gating mutations
- Peripheral immune cell abnormalities associated with cystic fibrosis
- Overexpression of RANK and M-CSFR in Monocytes of G551D-Bearing Patients with Cystic Fibrosis.
- Differential expression of genes and receptors in monocytes from patients with cystic fibrosis.
- Cellular metabolism and the NLRP3 inflammasome as therapeutic targets in cystic fibrosis
- Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function
- Animal Models in the Pathophysiology of Cystic Fibrosis
- Widespread alterations in the peripheral blood innate immune cell profile in cystic fibrosis reflect lung pathology
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