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Protease-activated receptor 1 in the pathogenesis of cystic fibrosis.
BMJ Open Respir Res
January 2025
Murdoch Children's Research Institute, Parkville, Victoria, Australia.
Background: The most common cause of death in those with cystic fibrosis (CF) is respiratory failure due to bronchiectasis resulting from repeated cycles of respiratory infection and inflammation. Protease-activated receptor 1 (PAR1) is a cell surface receptor activated by serine proteases including neutrophil elastase, which is recognised as a potent modulator of inflammation. While PAR1 is known to play an important role in regulating inflammation, nothing is known about any potential role of this receptor in CF pathogenesis.
View Article and Find Full Text PDFBackground & Aims: Intestinal tuft cells have recently been the interest of studies in several human gastrointestinal diseases. However, the impact of tuft cell deletion on intestinal physiological functions are not fully understood. This study investigated the effects of acute tuft cell loss on nutrient absorption and cell lineage differentiation.
View Article and Find Full Text PDFEnteric Duplication Cyst Associated With Meckel's Diverticulum: A Rare Cause of Acute Abdomen.
J Investig Med High Impact Case Rep
August 2024
Northern Adelaide Local Health Network, Haydown Road, Elizabeth Vale, South Australia.
We report a case of a 26-year-old type 1 diabetic woman presenting with acute lower abdominal pain, bloating, and vomiting. Initial examination revealed right lower quadrant tenderness and a suprapubic mass. Computed tomography (CT) imaging identified a 12-cm cystic structure suggestive of a dilated bowel loop with an adjacent inflamed 7-cm small bowel segment.
View Article and Find Full Text PDFIntestinal duplication in a miniature Schnauzer dog.
Can Vet J
August 2024
Ontario Veterinary College, University of Guelph, 50 Stone Road East, Guelph, Ontario N1G 2W1.
A 9-week-old miniature Schnauzer dog was brought to a veterinary clinic because of an acute onset of vomiting. A 2 to 3-centimeter, round, firm structure in the mid-abdomen was palpated with a repeatable pain response. An exploratory laparotomy revealed a grossly cystic-appearing mass on the distal ileum.
View Article and Find Full Text PDFAmelioration of airway and GI disease in G551D-CF ferrets by AAV1 and AAV6.
Gene Ther
September 2024
Departments of Physiology and Medicine, Johns Hopkins University, Baltimore, MD, USA.
Gene therapy for CF has concentrated on targeting the lung. Here we took a different approach by injecting into the cephalic vein and spraying into the trachea of G551D, CF ferrets either AAV1 or 6 containing Δ27-264-CFTR, a truncated version of CFTR. Treatment with the potentiator VX-770 was halted for 7 days before instillation to induce a disease phenotype.
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