PKD1 deficiency induces Bronchiectasis in a porcine ADPKD model.

ABSTRACT:

Background

Autosomal dominant polycystic kidney disease (ADPKD) is a prevalent genetic disorder, mainly characterized by the development of renal cysts, as well as various extrarenal manifestations. Previous studies have shown that ADPKD is related to bronchiectasis, while its pathogenic mechanism is unclear. In previous studies, we have generated the PKD1^+/- pigs to simulate the progression of cyst formation and physiological alterations similar to those seen in ADPKD patients.

Methods

Phenotypic changes to airway epithelial cell and mesenchymal cell in PKD1^+/- pigs were assessed by histological analysis. The molecular mechanisms driving these processes were investigated by using PKD1^+/- pig lungs, human mesenchymal cells, and generating PKD

SUBMITTER: Wang R 

PROVIDER: S-EPMC9617414 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature