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Molecule Shows Early Potential in Treating CF Due to F508del Mutation
An orally available small molecule called XL147 rescued the function of CFTR — the faulty protein in cystic fibrosis (CF) patients — in lab-grown airway cells from patients carrying F508del, the most common CF-causing mutation, a study shows. Adding XL147 to C18, a compound similar to the CFTR modulator lumacaftor (VX-809), also resulted in a […]The post Molecule Shows Early Potential in Treating CF Due to F508del Mutation appeared first on Cystic Fibrosis News Today
source http://hub.page/physical-disorders/molecule-shows-early-potential-in-treating-cf-due-to-f508del-mutation
source https://hubpage2.wordpress.com/2021/12/28/molecule-shows-early-potential-in-treating-cf-due-to-f508del-mutation/
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Novel therapy of bicarbonate, glutathione and ascorbic acid improves cystic fibrosis mucus transport.
PMID: Am J Respir Cell Mol Biol. 2020 May 6. Epub 2020 May 6. PMID: 32374624 Abstract Title: Novel Therapy of Bicarbonate, Glutathione and Ascorbic Acid Improves Cystic Fibrosis Mucus Transport. Abstract: Defective airway mucus clearance is a defining characteristic of CF lung disease, and improvements to current mucolytic strategies are needed. Novel approaches targeting a range of contributing mechanisms are in various stages of preclinical and clinical development. ARINA-1 is a new, nebulized product comprised of ascorbic acid, glutathione, and bicarbonate. Using micro-optical coherence tomography, we tested the effect of ARINA-1 on central features of mucociliary clearance in F508del/F508del primary human bronchial epithelial cells to assess its potential as a mucoactive therapy in CF. We found that ARINA-1 significantly augmented mucociliary transport (MCT) rates, both alone and with CFTR modulator therapy, whereas airway hydration and ciliary beating were largely unchanged compared to PBS vehicle control. Analysis of mucus reflectivity and particle tracking microrheology indicated that ARINA-1 restores mucus clearance by principally reducing mucus layer viscosity. The combination of bicarbonate and glutathione elicited increases to MCT rate comparable to those seen with ARINA-1, indicating the importance of this interaction to the impact of ARINA-1 on mucus transport; this effect was not recapitulated with bicarbonate alone or bicarbonate combined with ascorbic acid. Assessment of CFTR chloride transport revealed an increase in CFTR-mediated chloride secretion in response to ARINA-1 in CFBE41o- cells expressing wild-type CFTR, driven by CFTR activity stimulation by ascorbate. This response was absent in CFBE41o- F508del cells treated with VX-809 and primary HBE cells, implicating CFTR-independent mechanisms for the effect of ARINA-1 on CF mucus. Together, these studies indicate that ARINA-1 is a novel potential therapy for the treatment of impaired mucus clearance in CF.
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Glasgow > | Third Party Story Exposes Some Un-Answered Queries About VX-809
...Meyer Christensen. All of us give thanks to also Vivien McGrath (Glasgow) and Andrew Beare (Copenhagen) pertaining to editorial guidance. Last but not least, many of us gratefully acknowledge...
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