Inhibition of pannexin-1 does not restore electrolyte balance in precystic Pkd1 knockout mice.

ABSTRACT: Mutations in PKD1 and PKD2 cause autosomal dominant polycystic kidney disease (ADPKD), which is characterized by the formation of fluid-filled cysts in the kidney. In a subset of ADPKD patients, reduced blood calcium (Ca²⁺) and magnesium (Mg²⁺) concentrations are observed. As cystic fluid contains increased ATP concentrations and purinergic signaling reduces electrolyte reabsorption, we hypothesized that inhibiting ATP release could normalize blood Ca²⁺ and Mg²⁺ levels in ADPKD. Inducible kidney-specific Pkd1 knockout mice (iKsp-Pkd1^-/-) exhibit hypocalcemia and hypomagnesemia in a precystic stage and show increased expression of the ATP-release channel pannexin-1. Therefore, we administered the pannexin-1 inhibitor brilliant blue-FCF

SUBMITTER: van Megen WH 

PROVIDER: S-EPMC10984814 | biostudies-literature | 2024 Apr

REPOSITORIES: biostudies-literature