Open AccessThe <b><i>von Hippel-Lindau</i></b> Gene Is Required to Maintain Renal Proximal Tubule and Glomerulus Integrity in Zebrafish Larvae
Author(s) -
Ellen van Rooijen,
Glenn van de Hoek,
Ive Logister,
Henry Ajzenberg,
Nine V A M Knoers,
Freek van Eeden,
Emile E. Voest,
Stefan SchulteMerker,
Rachel Giles
Publication year - 2018
Publication title -
the nephron journals/nephron journals
Language(s) - English
Resource type - Journals
eISSN - 2235-3186
pISSN - 1660-8151
DOI - 10.1159/000484096
Subject(s) - zebrafish , pronephros , biology , glomerulus , microbiology and biotechnology , endocytic cycle , von hippel–lindau disease , pathology , convoluted tubule , kidney , endocrinology , endocytosis , medicine , cell , genetics , gene , disease
von Hippel-Lindau (VHL) disease is characterized by the development of benign and malignant tumours in many organ systems, including renal cysts and clear cell renal cell carcinoma. It is not completely understood what underlies the development of renal pathology, and the use of murine Vhl models has been challenging due to limitations in disease conservation. We previously described a zebrafish model bearing inactivating mutations in the orthologue of the human VHL gene.