Integrin alpha1 beta1 promotes interstitial fibrosis in a mouse model of polycystic kidney disease.

ABSTRACT: Fibrosis is the cause of end-stage kidney failure in patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD). The molecular and cellular mechanisms involved in fibrosis are complex and anti-fibrotic therapies have so far failed to make an impact on patient welfare. Using unbiased proteomics analysis on the Pkd1 ^nl/nl mouse, we found that expression of the integrin α1 subunit is increased in this model of ADPKD. In human ADPKD tissue and two single cell RNA kidney disease datasets, ITGA1 was also upregulated. To investigate the functional role of this integrin subunit in ADPKD, we generated a Pkd1 ^nl/nl Itga1 ^-/- mouse. We observed a significant reduction in kidney volume and kidney dysfunction in mice lacking the integ

SUBMITTER: Grenier C 

PROVIDER: S-EPMC11526950 | biostudies-literature | 2024 Oct

REPOSITORIES: biostudies-literature