Association of cystic fibrosis transmembrane conductance regulator with epithelial sodium channel subunits carrying Liddle's syndrome mutations.

The association of the cystic fibrosis transmembrane conductance regulator (CFTR) and epithelial sodium channel (ENaC) in the pathophysiology of cystic fibrosis (CF) is controversial. Previously, we demonstrated a close physical association between wild-type (WT) CFTR and WT ENaC. We have also shown that the F508del CFTR fails to associate with ENaC unless the mutant protein is rescued pharmacologically or by low temperature.

Relationship between lung function impairment and health-related quality of life in COPD and interstitial lung disease.

Background: Health-related quality-of-life (HRQL) measures have been correlated with lung function in patients with COPD and interstitial lung disease (ILD). However, different pathophysiologic mechanisms may influence how these distinct diseases affect HRQL, resulting in differing HRQL by pulmonary diagnosis among patients with similar severity of ventilatory impairment.

Methods: The National Heart, Lung, and Blood Institute Lung Tissue Research Consortium provided data on well-characterized participants with COPD (n = 576) and ILD (n = 405) at four clinical sites.

Low temperature and chemical rescue affect molecular proximity of DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) and epithelial sodium channel (ENaC).

An imbalance of chloride and sodium ion transport in several epithelia is a feature of cystic fibrosis (CF), an inherited disease that is a consequence of mutations in the cftr gene. The cftr gene codes for a Cl(-) channel, the cystic fibrosis transmembrane conductance regulator (CFTR). Some mutations in this gene cause the balance between Cl(-) secretion and Na(+) absorption to be disturbed in the airways; Cl(-) secretion is impaired, whereas Na(+) absorption is elevated.

The unfolded protein response (UPR)-activated transcription factor X-box-binding protein 1 (XBP1) induces microRNA-346 expression that targets the human antigen peptide transporter 1 (TAP1) mRNA and governs immune regulatory genes.

To identify endoplasmic reticulum (ER) stress-induced microRNAs (miRNA) that govern ER protein influx during the adaptive phase of unfolded protein response, we performed miRNA microarray profiling and analysis in human airway epithelial cells following ER stress induction using proteasome inhibition or tunicamycin treatment. We identified miR-346 as the most significantly induced miRNA by both classic stressors. miR-346 is encoded within an intron of the glutamate receptor ionotropic delta-1 gene (GRID1), but its ER stress-associated expression is independent of GRID1.

Chondrocyte death by apoptosis is associated with the initiation and severity of articular cartilage degradation.

Aim: To investigate the role of chondrocyte apoptosis in the initiation and severity of articular cartilage (AC) damage.

Methods: Articular cartilage from equine metacarpophalangeal (MCP) (n = 13) and metatarsophalangeal (MTP) (n = 16) joints was used and each graded macroscopically for cartilage degradation (macroscopic osteoarthritis [OA] grade). Cartilage was sampled from six regions on the articular surface of both joint types and graded using a 'modified' Mankin scoring system.

Molecular proximity of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel assessed by fluorescence resonance energy transfer.

We present the evidence for a direct physical association of cystic fibrosis transmembrane conductance regulator (CFTR) and epithelial sodium channel (ENaC), two major ion channels implicated in the pathophysiology of cystic fibrosis, a devastating inherited disease. We employed fluorescence resonance energy transfer, a distance-dependent imaging technique with capability to detect molecular complexes with near angstrom resolution, to estimate the proximity of CFTR and ENaC, an essential variable for possible physical interaction to occur. Fluorescence resonance energy transfer studies were complemented with a classic biochemical approach: coimmunoprecipitation.

Effect of trotting velocity on work patterns of the hind limbs of Greyhounds.

Objective: To quantify the effects of trotting velocity on joint angular excursions, net joint moments, and powers across the hind limb joints in Greyhounds.

Animals: 5 healthy Greyhounds with no history of lameness of the hind limbs.

Procedures: Small reflective markers were applied to the skin over the joints of the hind limbs, and a 4-camera kinematic system was used to record positional data at 200 Hz in tandem with force platform data while the dogs trotted on a runway at slow, medium, and fast velocities.

Microscopic change in macroscopically normal equine cartilage from osteoarthritic joints.

The objective of this study was to assess whether macroscopically normal articular cartilage taken from joints containing focal osteoarthritic lesions is histologically similar to articular cartilage taken from macroscopically normal joints. Metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints were obtained from 10 horses following euthanasia. Gross articular cartilage damage was scored and the cartilage assigned to one of two groups: (1) macroscopically normal cartilage from normal joints (control) and (2) macroscopically normal cartilage from diseased joints in which there were focal osteoarthritic lesions.

Distribution of power across the hind limb joints in Labrador Retrievers and Greyhounds.

Objective: To quantify angular excursions; net joint moments; and powers across the stifle, tarsal, and metatarsophalangeal (MTP) joints in Labrador Retrievers and Greyhounds and investigate differences in joint mechanics between these 2 breeds of dogs.

Animals: 12 clinically normal dogs (6 Greyhounds and 6 Labrador Retrievers) with no history of hind limb lameness.

Procedure: Small retroreflective markers were applied to the skin over the pelvic limb joints, and a 4-camera kinematic system captured data at 200 Hz in tandem with force platform data while the dogs trotted on a runway.