Skip to main content
U.S. Department of Energy
Office of Scientific and Technical Information

A highly selective, cell-permeable furin inhibitor BOS-318 rescues key features of cystic fibrosis a

Journal Article · · Cell Chem. Biol.

Research Organization:
Advanced Photon Source (APS), Argonne National Laboratory (ANL), Argonne, IL (US)
Sponsoring Organization:
DOE - Office Of Science; INDUSTRY
OSTI ID:
1900699
Journal Information:
Cell Chem. Biol., Journal Name: Cell Chem. Biol. Journal Issue: (6) Vol. 29
Country of Publication:
United States
Language:
ENGLISH

References (49)

Proprotein convertase inhibition promotes ciliated cell differentiation - a potential mechanism for the inhibition of Notch1 signalling by decanoyl-RVKR-chloromethylketone journal November 2016
Evidence for airway surface dehydration as the initiating event in CF airway disease journal January 2007
Role of exotoxin A in inducing severe Pseudomonas aeruginosa infections in mice journal September 1995
Acute Regulation of the Epithelial Sodium Channel in Airway Epithelia by Proteases and Trafficking journal December 2010
Reduced Sodium Transport With Nasal Administration of the Prostasin Inhibitor Camostat in Subjects With Cystic Fibrosis journal July 2013
The biology and therapeutic targeting of the proprotein convertases journal April 2012
PHENIX: a comprehensive Python-based system for macromolecular structure solution journal January 2010
The proprotein convertase furin in tumour progression journal May 2017
Mucus clearance as a primary innate defense mechanism for mammalian airways journal March 2002
Distinct Pools of Epithelial Sodium Channels Are Expressed at the Plasma Membrane journal November 2004
Furin at the cutting edge: From protein traffic to embryogenesis and disease journal October 2002
Phaser crystallographic software journal July 2007
Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. journal November 1986
Notch1 Autoactivation via Transcriptional Regulation of Furin, Which Sustains Notch1 Signaling by Processing Notch1-Activating Proteases ADAM10 and Membrane Type 1 Matrix Metalloproteinase journal November 2015
Contribution of toxin A and elastase to virulence of Pseudomonas aeruginosa in chronic lung infections of rats journal June 1982
The Epithelial Sodium Channel Is a Modifier of the Long-Term Nonprogressive Phenotype Associated with F508del CFTR Mutations journal December 2017
Pulmonary Epithelial Sodium-Channel Dysfunction and Excess Airway Liquid in Pseudohypoaldosteronism journal July 1999
A Multibasic Cleavage Site in the Spike Protein of SARS-CoV-2 Is Essential for Infection of Human Lung Cells journal May 2020
Cell entry mechanisms of SARS-CoV-2 journal May 2020
Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis journal July 2014
Epithelial Sodium Channels Are Activated by Furin-dependent Proteolysis journal April 2004
Processing of Pseudomonas exotoxin by a cellular protease results in the generation of a 37,000-Da toxin fragment that is translocated to the cytosol journal November 1990
Therapeutic uses of furin and its inhibitors: a patent review journal January 2015
The Relative Roles of Passive Surface Forces and Active Ion Transport in the Modulation of Airway Surface Liquid Volume and Composition journal July 2001
Tgf-β1 Inhibits Cftr Biogenesis and Prevents Functional Rescue of ΔF508-Cftr in Primary Differentiated Human Bronchial Epithelial Cells journal May 2013
The spike glycoprotein of the new coronavirus 2019-nCoV contains a furin-like cleavage site absent in CoV of the same clade journal April 2020
Loss of furin cleavage site attenuates SARS-CoV-2 pathogenesis journal January 2021
The Notch1 receptor is cleaved constitutively by a furin-like convertase journal July 1998
Coot model-building tools for molecular graphics journal November 2004
Kinetics of the reversible inhibition of enzyme-catalysed reactions by tight-binding inhibitors journal August 1969
Epithelial Na+ Channels Are Fully Activated by Furin- and Prostasin-dependent Release of an Inhibitory Peptide from the γ-Subunit journal March 2007
Modulation of the Epithelial Sodium Channel (ENaC) by Bacterial Metalloproteases and Protease Inhibitors journal June 2014
BacMam production and crystal structure of nonglycosylated apo human furin at 1.89 Å resolution journal March 2019
FurinDB: A Database of 20-Residue Furin Cleavage Site Motifs, Substrates and Their Associated Drugs journal February 2011
X-ray Structures of the Proprotein Convertase Furin Bound with Substrate Analogue Inhibitors Reveal Substrate Specificity Determinants beyond the S4 Pocket journal January 2018
Neutrophil elastase activates near-silent epithelial Na+channels and increases airway epithelial Na+transport journal May 2005
Elevated furin levels in human cystic fibrosis cells result in hypersusceptibility to exotoxin A–induced cytotoxicity journal November 2007
Furin Inhibitors Block SARS-CoV-2 Spike Protein Cleavage to Suppress Virus Production and Cytopathic Effects journal October 2020
Production of elastase, exotoxin A, and alkaline protease in sputa during pulmonary exacerbation of cystic fibrosis in patients chronically infected by Pseudomonas aeruginosa journal April 1995
Development of cystic fibrosis and noncystic fibrosis airway cell lines journal May 2003
A Selective Irreversible Inhibitor of Furin Does Not Prevent Pseudomonas Aeruginosa Exotoxin A-Induced Airway Epithelial Cytotoxicity journal July 2016
Camostat Attenuates Airway Epithelial Sodium Channel Function in Vivo through the Inhibition of a Channel-Activating Protease journal February 2009
Inhibition of Protease–Epithelial Sodium Channel Signaling Improves Mucociliary Function in Cystic Fibrosis Airways journal September 2016
Emerging Concepts and Therapies for Mucoobstructive Lung Disease journal November 2018
Regulation of Airway Surface Liquid Volume and Mucus Transport by Active Ion Transport journal January 2004
CFTR as a cAMP-Dependent Regulator of Sodium Channels journal August 1995
Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis journal July 2002
X-ray Structures of Human Furin in Complex with Competitive Inhibitors journal April 2014
A Segment of γ ENaC Mediates Elastase Activation of Na+ Transport journal November 2007

Similar Records

Intestinal permeability to (/sup 51/Cr)EDTA in children with cystic fibrosis
Journal Article · 1986 · J. Pediatr. Gastroenterol. Nutr.; (United States) · OSTI ID:5019752

Scoliosis in cystic fibrosis - an appraisal
Journal Article · 1982 · Skelet. Radiol.; (United States) · OSTI ID:5029738

STUDIES ON BASIC CHARGED MOLECULES AND CELL MEMBRANES IN CYSTIC FIBROSIS.
Journal Article · 1970 · Proc. Soc. Exp. Biol. Med. 137: No. 4, 1283-8(Sep 1971). · OSTI ID:4737307

Related Subjects