Combined effects of VX-770 and VX-809 on several functional abnormalities of F508del-CFTR channels [PDF]
BackgroundThe most common cystic fibrosis-associated mutation, the deletion of phenylalanine 508 (F508del), results in channels with poor membrane expression and impaired function.
Kopeikin, Z. +3 more
core +3 more sources
Toxicokinetics of (±)-VX in hairless guinea pigs and marmosets - in vitro metabolism of (±)-VX
Although the toxicokinetics of several nerve gases, such as sarin and soman have been thoroughly investigated, the toxicokinetics of (±)-VX are still poorly understood.
Wiel, H.J. van der +5 more
core +2 more sources
Mislocalization of CFTR expression in acute pancreatitis and the beneficial effect of VX‐661 + VX‐770 treatment on disease severity [PDF]
Cystic fibrosis transmembrane conductance regulator (CFTR) is an important ion channel in epithelial cells. Its malfunction has several serious consequences, like developing or aggravating acute pancreatitis (AP).
Lóránd Kiss +24 more
core +2 more sources
Russian VX: Inhibition and Reactivation of Acetylcholinesterase Compared with VX Agent [PDF]
Abstract: Organophosphorus compounds such as nerve agents inhibit, practically irreversibly, cholinesterases by their phosphorylation in the active site of these enzymes. Current antidotal treatment used in the case of acute nerve agent intoxications consists of combined administration of anticholinergic drug (usually atropine) and acetylcholinesterase
Kamil, Kuca +5 more
openaire +2 more sources
Clinical activity of a htert (vx-001) cancer vaccine as post-chemotherapy maintenance immunotherapy in patients with stage IV non-small cell lung cancer: final results of a randomised phase 2 clinical trial [PDF]
The cancer vaccine Vx-001, which targets the universal tumour antigen TElomerase Reverse Transcriptase (TERT), can mount specific Vx-001/TERT CD8 + cytotoxic T cells; this immune response is associated with improved overall survival (OS) in patients with
Syrigos, Kostantinos N. +60 more
core +1 more source
Theratyping of the Rare CFTR Genotype A559T in Rectal Organoids and Nasal Cells Reveals a Relevant Response to Elexacaftor (VX-445) and Tezacaftor (VX-661) Combination [PDF]
Despite the promising results of new CFTR targeting drugs designed for the recovery of F508del- and class III variants activity, none of them have been approved for individuals with selected rare mutations, because uncharacterized CFTR variants lack ...
Kleinfelder K. +7 more
core +3 more sources
Mechanism-based corrector combination restores ΔF508-CFTR folding and function. [PDF]
The most common cystic fibrosis mutation, Delta F508 in nucleotide binding domain 1 (NBD1), impairs cystic fibrosis transmembrane conductance regulator (CFTR)-coupled domain folding, plasma membrane expression, function and stability. VX-809, a promising
Okiyoneda, T. +21 more
core +1 more source
VX toxicity in the Göttingen minipig [PDF]
The present experiments determined the intramuscular LD50 of VX in male Göttingen minipigs at two stages of development. In pubertal animals (115 days old), the LD50 of VX was indeterminate, but approximated 33.3μg/kg. However, in sexually mature animals (152 days old), the LD50 was estimated to be only 17.4μg/kg.
Jeffrey L, Langston, Todd M, Myers
openaire +2 more sources
The Pulsation Spectrum of VX Hydrae [PDF]
14 pages, 5 figures, published in Publications of the Astronomical Society of the Pacific, v.121, p.1076 (October 2009)
Templeton, M. R. +5 more
openaire +2 more sources
Treatment of Polarized Cystic Fibrosis Airway Cells With HGF Prevents VX-661-Rescued F508del-CFTR Destabilization Caused by Prolonged Co-exposure to VX-770 [PDF]
Cystic fibrosis (CF), the most common inherited disease in Caucasians, is caused by mutations in CFTR, the most frequent of which is F508del. F508del causes ER retention and degradation of the mutant CFTR protein, but also defective channel gating and
Matos, Ana M. +2 more
core +1 more source

