Characterization of mitochondrial function in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function

Airway surface liquid homeostasis in cystic fibrosis: pathophysiology and therapeutic targets

(2015) Iram J Haq et al.   THORAX

Targeted therapies to improve CFTR function in cystic fibrosis

(2015) Malcolm Brodlie et al.   Genome Medicine

Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells

(2014) Maria Favia et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Glycolytic enzyme upregulation and numbness of mitochondrial activity characterize the early phase of apoptosis in cerebellar granule cells

(2014) A. Bobba et al.   APOPTOSIS

Disruption of Interleukin-1β Autocrine Signaling Rescues Complex I Activity and Improves ROS Levels in Immortalized Epithelial Cells with Impaired Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Function

(2014) Mariángeles Clauzure et al.   PLoS One

Dissecting the molecular mechanism by which NH2htau and Aβ1-42 peptides impair mitochondrial ANT-1 in Alzheimer disease

(2013) A. Bobba et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mitochondrial respiratory chain Complexes I and IV are impaired by β-amyloid via direct interaction and through Complex I-dependent ROS production, respectively

(2013) A. Bobba et al.   MITOCHONDRION

VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1

(2013) Hong Yu Ren et al.   MOLECULAR BIOLOGY OF THE CELL

The CFTR Ion Channel: Gating, Regulation, and Anion Permeation

(2013) T.-C. Hwang et al.   Cold Spring Harbor Perspectives in Medicine

Targeting mitochondrial dysfunction in lung diseases: emphasis on mitophagy

(2013) Angara Sureshbabu et al.   Frontiers in Physiology

CFTR activity and mitochondrial function

(2013) Angel Gabriel Valdivieso et al.   Redox Biology

Redox regulation of mitochondrial function with emphasis on cysteine oxidation reactions

(2013) Ryan J. Mailloux et al.   Redox Biology

Amyloid-β Peptide Binds to Cytochrome C Oxidase Subunit 1

(2012) Luis Fernando Hernandez-Zimbron et al.   PLoS One

GSH monoethyl ester rescues mitochondrial defects in cystic fibrosis models

(2011) M. Kelly-Aubert et al.   HUMAN MOLECULAR GENETICS

There is no evidence that mitochondria are the main source of reactive oxygen species in mammalian cells

(2011) Guy C. Brown et al.   MITOCHONDRION

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809

(2011) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Superoxide Flashes in Mouse Skeletal Muscle Are Produced by Discrete Arrays of Active Mitochondria Operating Coherently

(2010) Sandrine Pouvreau   PLoS One

Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease

(2009) F. Distelmaier et al.   BRAIN