XB-ART-51813Semin Cell Dev Biol March 1, 2016; 51 117-24.
Using Xenopus to study genetic kidney diseases.
Modern sequencing technology is revolutionizing our knowledge of inherited kidney disease. However, the molecular role of genes affected by the rapidly rising number of identified mutations is lagging behind. Xenopus is a highly useful, but underutilized model organism with unique properties excellently suited to decipher the molecular mechanisms of kidney development and disease. The embryonic kidney (pronephros) can be manipulated on only one side of the animal and its formation observed directly through the translucent skin. The moderate evolutionary distance between Xenopus and humans is a huge advantage for studying basic principles of kidney development, but still allows us to analyze the function of disease related genes. Optogenetic manipulations and genome editing by CRISPR/Cas are exciting additions to the toolbox for disease modelling and will facilitate the use of Xenopus in translational research. Therefore, the future of Xenopus in kidney research is bright.
PubMed ID: 26851624
Article link: Semin Cell Dev Biol
Genes referenced: ace agt anks6 bmp4 eya1 foxc1 hnf1b invs lmx1b.1 nek8 nphp3 pax2 pkd2 six1 six2 sox17a tsc1 wdpcp wnt4 wt1
Disease Ontology terms: CAKUT
Article Images: [+] show captions
|Fig. 1. Schematic of key developmental events during Xenopus pronephros development and the embryonic stages at which they are in full progress. The tissues labeled in red correspond to the lateral plate mesoderm and subsequent tissues where renal specification and further development occurs. MET: mesenchymal to epithelial transition, hpf: hours post fertilization, NF: Nieuwkoop–Faber stage.|
|Fig. 2. Changes to renal morphology resulting from inherited kidney diseases. The white circles represent renal cysts and are typically small and positioned at the cortico-medullary border in nephronophthisis, and large and ubiquitous in autosomal dominant polycystic kidney disease. Examples of renal malformations observed in CAKUT (congenital anomalies of the kidney and urinary tract) patients are depicted.|