Mitochondria in lung disease

SM Cloonan, AMK Choi - The Journal of clinical …, 2016 - Am Soc Clin Investig
Mitochondria are a distinguishing feature of eukaryotic cells. Best known for their critical
function in energy production via oxidative phosphorylation (OXPHOS), mitochondria are …

Mitochondrial dysfunction and damage associated molecular patterns (DAMPs) in chronic inflammatory diseases

CSD Cruz, MJ Kang - Mitochondrion, 2018 - Elsevier
Inflammation represents a comprehensive host response to external stimuli for the purpose
of eliminating the offending agent, minimizing injury to host tissues and fostering repair of …

Cystic fibrosis, CFTR, and colorectal cancer

P Scott, K Anderson, M Singhania… - International journal of …, 2020 - mdpi.com
Cystic fibrosis (CF), caused by biallelic inactivating mutations in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene, has recently been categorized as a …

[HTML][HTML] Peripheral muscle abnormalities in cystic fibrosis: Etiology, clinical implications and response to therapeutic interventions

M Gruet, T Troosters, S Verges - Journal of Cystic Fibrosis, 2017 - Elsevier
Peripheral muscle dysfunction is an important systemic consequence of cystic fibrosis (CF)
with major clinical implications, such as exercise intolerance and reduced quality of life …

The distribution and role of the CFTR protein in the intracellular compartments

A Lukasiak, M Zajac - Membranes, 2021 - mdpi.com
Cystic fibrosis is a hereditary disease that mainly affects secretory organs in humans. It is
caused by mutations in the gene encoding CFTR with the most common phenylalanine …

Cystic Fibrosis of the Pancreas: The Role of CFTR Channel in the Regulation of Intracellular Ca2+ Signaling and Mitochondrial Function in the Exocrine Pancreas

T Madácsy, P Pallagi, J Maleth - Frontiers in Physiology, 2018 - frontiersin.org
Cystic fibrosis (CF) is the most common genetic disorder that causes a significant damage in
secretory epithelial cells due to the defective ion flux across the cystic fibrosis …

The chloride anion as a signalling effector

ÁG Valdivieso, TA Santa‐Coloma - Biological Reviews, 2019 - Wiley Online Library
The specific role of the chloride anion (Cl−) as a signalling effector or second messenger
has been increasingly recognized in recent years. It could represent a key factor in the …

Elexacaftor–Tezacaftor–Ivacaftor improves exercise capacity in adolescents with cystic fibrosis

AJ Causer, JK Shute, MH Cummings… - Pediatric …, 2022 - Wiley Online Library
Abstract Objective Elexacaftor/Tezacaftor/Ivacaftor is a cystic fibrosis transmembrane
conductance regulator (CFTR) modulator with the potential to improve exercise capacity …

[HTML][HTML] Circulating biomarkers of antioxidant status and oxidative stress in people with cystic fibrosis: A systematic review and meta-analysis

AJ Causer, JK Shute, MH Cummings, AI Shepherd… - Redox biology, 2020 - Elsevier
Introduction Oxidative stress may play an important role in the pathophysiology of cystic
fibrosis (CF). This review aimed to quantify CF-related redox imbalances. Methods …

[HTML][HTML] Whole-blood transcriptomic responses to lumacaftor/ivacaftor therapy in cystic fibrosis

BT Kopp, J Fitch, L Jaramillo, CL Shrestha… - Journal of Cystic …, 2020 - Elsevier
Background Cystic fibrosis (CF) remains without a definitive cure. Novel therapeutics
targeting the causative defect in the cystic fibrosis transmembrane conductance regulator …