Osteoblast CFTR inactivation reduces differentiation and osteoprotegerin expression in a mouse model of cystic fibrosis-related bone disease. [PDF]
PLoS ONE, 2013 Low bone mass and increased fracture risk are recognized complications of cystic fibrosis (CF). CF-related bone disease (CFBD) is characterized by uncoupled bone turnover--impaired osteoblastic bone formation and enhanced osteoclastic bone resorption ...
Michael S Stalvey +6 more
doaj +1 more source
Integrating Human Intestinal Organoids into FDA'S New Approach Methodologies for Drug Discovery
Advanced Science, EarlyView.Illustration summarizes how human intestinal organoids (HIOs) are becoming transformative in preclinical research. Preclinical drug discovery pipelines often rely on animal models for ADMET studies, even though interspecies ADME gaps, poor external validity, and high attrition rates are common.
Debarun Patra +6 more
wiley +1 more source
CFTR Functions as a Tumor Suppressor and Is Regulated by DNA Methylation in Colorectal Cancer
Cancer Management and Research, 2020 Can Liu,1 Chao Song,2 Jiaxi Li,3 Qing Sun1,3 1Department of Pathology, Shandong Provincial Qianfoshan Hospital, Cheeloo College of Medicine, Shandong University, Jinan, Shandong Province, People’s Republic of China; 2Department of Pathology, Zibo ...
Liu C, Song C, Li J, Sun Q
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Proteomic identification of calumenin as a G551D-CFTR associated protein. [PDF]
PLoS ONE, 2012 Cystic fibrosis (CF) is the most common lethal autosomal recessive disease in the Caucasian population. It is due to mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Ling Teng +7 more
doaj +1 more source
Frontiers in Pharmacology, 2021 Decreased human epididymis protein 4 (HE4) plasma levels were reported in cystic fibrosis (CF) patients under CFTR potentiator ivacaftor therapy, which inversely correlated with lung function improvement.
Zsolt Bene +15 more
doaj +1 more source
Soft Artificial Ciliary Brush with Integrated Haptic Feedback for Efficient Airway Mucus Cleaning
Advanced Intelligent Systems, EarlyView.A fundamental mucus cleaning mechanism is reported, which utilizes both artificial cilia mechanical sweeping and suction‐based mucus cleaning. A novel haptic feedback interface for the artificial cilia provides touch feedback on tissue contact, enhancing operator control and safety.
Zhongming Lyu +7 more
wiley +1 more source
Efficacy and Mechanism Evaluation, 2016 Background: Cystic fibrosis (CF) is a chronic, life-limiting disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene leading to abnormal airway surface ion transport, chronic lung infections, inflammation and eventual ...
Eric WFW Alton +80 more
doaj +1 more source
Intra-amniotic delivery of CFTR-expressing adenovirus does not reverse cystic fibrosis phenotype in inbred CFTR-knockout mice [PDF]
, 2008 This article is available open access through the publisher’s website at the link below. Copyright © 2008 The American Society of Gene Therapy.Due to its early onset and severe prognosis, cystic fibrosis (CF) has been suggested as a candidate disease for
A Bergau +59 more
core +1 more source
Ubiquitination and degradation of CFTR by the E3 ubiquitin ligase MARCH2 through its association with adaptor proteins CAL and STX6. [PDF]
PLoS ONE, 2013 Golgi-localized cystic fibrosis transmembrane conductance regulator (CFTR)-associated ligand (CAL) and syntaxin 6 (STX6) regulate the abundance of mature, post-ER CFTR by forming a CAL/STX6/CFTR complex (CAL complex) that promotes CFTR degradation in ...
Jie Cheng, William Guggino
doaj +1 more source
Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity. [PDF]
, 2019 Deletion of phenylalanine 508 (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel is the most frequent mutation causing cystic fibrosis (CF). F508del-CFTR is misfolded and prematurely degraded. Recently thymosin a-1 (
Armirotti, Andrea +13 more
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