Functional correction of renal defects in a mouse model for ARPKD through expression of the cloned wild-type Tg737 cDNA. Kidney Int 1996 Oct;50(4):1240-8
Date
10/01/1996Pubmed ID
8887283DOI
10.1038/ki.1996.433Scopus ID
2-s2.0-0029842984 (requires institutional sign-in at Scopus site) 34 CitationsAbstract
Autosomal recessive polycystic kidney disease (ARPKD) is characterized by the formation of large collecting tubule and ductular cysts that often result in renal insufficiency within the first decade of life. Understanding the process leading to cyst formation will require the identification and characterization of genes involved in the etiology of this disease. In this regard, we previously described the generation of a mouse model (TgN737Rpw) for ARPKD and the cloning of a candidate gene. Here we show direct involvement of the Tg737 gene in collecting duct cyst formation by expressing the wild-type Tg737 cDNA as a transgene in TgN737Rpw mutants. In contrast to TgN737Rpw mutants, the "rescued" animals survive longer, have normal renal function and normal localization of the EGFr to the basolateral surfaces of collecting duct epithelium.
Author List
Yoder BK, Richards WG, Sommardahl C, Sweeney WE, Michaud EJ, Wilkinson JE, Avner ED, Woychik RPAuthor
Ellis D. Avner MD Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AnimalsBlotting, Southern
Body Weight
DNA, Complementary
Disease Models, Animal
ErbB Receptors
Genetic Therapy
Genotype
Kidney
Kidney Concentrating Ability
Lectins
Mice
Mice, Mutant Strains
Plant Lectins
Polycystic Kidney, Autosomal Recessive
Protein Biosynthesis
Proteins
Time Factors
Transgenes
Tumor Suppressor Proteins
