Mutation of hepatocyte nuclear factor–1β inhibits Pkhd1 gene expression and produces renal cysts in mice

Abstract

Nonstandard abbreviations used: aquaporin-2 (AQP2); autosomal recessive polycystic kidney disease (ARPKD); bacterial artificial chromosome (BAC); blood urea nitrogen (BUN); cyanine 3 (Cy3); deoxyribonuclease I (DNase I); dominant-negative HNF-1 (DN-HNF1); electrophoretic mobility shift assay (EMSA); hepatocyte nuclear factor-1 (HNF-1); Lotus tetragonolobus agglutinin (LTA); maturity-onset diabetes mellitus of the young, type 5 (MODY5); mouse inner medullary collecting duct