Targeted inactivation of fh1 causes proliferative renal cyst development and activation of the hypoxia pathway.

Abstract

Germline mutations in the fumarate hydratase (FH) tumor suppressor gene predispose to leiomyomatosis, renal cysts, and renal cell cancer (HLRCC). HLRCC tumors overexpress HIF1alpha and hypoxia pathway genes. We conditionally inactivated mouse Fh1 in the kidney. Fh1 mutants developed multiple clonal renal cysts that overexpressed Hif1alpha and Hif2alpha. Hif… (More)

Topics

Figures and Tables

Sorry, we couldn't extract any figures or tables for this paper.