A knock-in mouse line conditionally expressing the tumor suppressor WTX/AMER1

Archive ouverte

Boutet, Agnès | Comai, Glenda | Charlet, Aurélie | Jian Motamedi, Fariba | Dhib, Haroun | Bandiera, Roberto | Schedl, Andreas

Edité par CCSD ; Wiley-Blackwell -

International audience. WTX/AMER1 is an important developmental regulator, mutations in which have been identified in a proportion of patients suffering from the renal neoplasm Wilms' tumor and in the bone malformation syndrome Osteopathia Striata with Cranial Sclerosis (OSCS). Its cellular functions appear complex and the protein can be found at the membrane, within the cytoplasm and the nucleus. To understand its developmental and cellular function an allelic series for Wtx in the mouse is crucial. Whereas mice carrying a conditional knock out allele for Wtx have been previously reported, a gain-of-function mouse model that would allow studying the molecular, cellular and developmental role of Wtx is still missing. Here we describe the generation of a novel mouse strain that permits the conditional activation of WTX expression. Wtx fused to GFP was introduced downstream a stop cassette flanked by loxP sites into the Rosa26 locus by gene targeting. Ectopic WTX expression is reported after crosses with several Cre transgenic mice in different embryonic tissues. Further, functionality of the fusion protein was demonstrated in the context of a Wtx null allele.

Consulter en ligne

Suggestions

Du même auteur

The WTX/AMER1 gene family: evolution, signature and function

Archive ouverte | Boutet, Agnès | CCSD

International audience. Background: WTX is a novel gene mutated in a proportion of Wilms' tumors and in patients suffering from sclerosing bone dysplasia. On the molecular level WTX has been shown to act as an antag...

Retinoic acid signaling is directly activated in cardiomyocytes and protects mouse hearts from apoptosis after myocardial infarction

Archive ouverte | da Silva, Fabio | CCSD

Retinoic acid (RA) is an essential signaling molecule for cardiac development and plays a protective role in the heart after myocardial infarction (MI). In both cases, the effect of RA signaling on cardiomyocytes, the principle ce...

Sox11 gene disruption causes congenital anomalies of the kidney and urinary tract (CAKUT). Sox11 gene disruption causes congenital anomalies of the kidney and urinary tract (CAKUT): SOX11 and CAKUT

Archive ouverte | Neirijnck, Yasmine | CCSD

International audience. Congenital abnormalities of the kidney and the urinary tract (CAKUT) belong to the most common birth defectsin human, but the molecular basis for the majority of CAKUT patients remains unknow...

Chargement des enrichissements...