The major goal of the Mouse and Cell Line Core is to provide Center investigators with common set of unique reagents and services to enable them to achieve the highest levels of insight into the problems in the PKD field. A secondary goal of the Core is to make these same reagents available to the research community at large. The Core reagents are and very comprehensive array of PKD-related animal models and well characterized mouse kidney cell lines with Pkd1 and Pkd2 mutations. The service provided by the Core is the expert consultation and collaboration to allow all Center investigators use these reagents to highest effect.
The specific aims of the Core are: 1. To make available a comprehensive array of animal models relevant to the study of polycystic kidney and liver disease. 2. To characterize and make available specialized mouse-derived cell lines with fixed and inducible mutations in Pkd1 and Pkd2. 3. To perform the necessary mouse breeding and genotyping to obtain experimental animals. 4. To prepare tissues from experimental animals for imaging-based analysis. 5. To provide expert instruction to Center investigators in the design and study of new animal models. 6. To make mouse and cell line models available to the research community worldwide. The state-of-the-art in mouse models of human disease has moved from simple knockouts to complex combinations of conditional alleles with inducible transgenes. Issues of genetic background must always be borne in mind. New methods of BAG modification and transgenesis offer the ability to address very specific questions. All these approaches require expertise for optimal application. The Core and its PI will provide this service to all Center faculty as well as Center collaborators, thus facilitating and enhancing the respective projects.
| Li, Ao; Tian, Xin; Zhang, Xiaoli et al. (2015) Human polycystin-2 transgene dose-dependently rescues ADPKD phenotypes in Pkd2 mutant mice. Am J Pathol 185:2843-60 |
| Merrick, David; Bertuccio, Claudia A; Chapin, Hannah C et al. (2014) Polycystin-1 cleavage and the regulation of transcriptional pathways. Pediatr Nephrol 29:505-11 |
| Cai, Yiqiang; Fedeles, Sorin V; Dong, Ke et al. (2014) Altered trafficking and stability of polycystins underlie polycystic kidney disease. J Clin Invest 124:5129-44 |
| Paavola, Jere; Schliffke, Simon; Rossetti, Sandro et al. (2013) Polycystin-2 mutations lead to impaired calcium cycling in the heart and predispose to dilated cardiomyopathy. J Mol Cell Cardiol 58:199-208 |
| Yuan, Shiaulou; Zhao, Lu; Sun, Zhaoxia (2013) Dissecting the functional interplay between the TOR pathway and the cilium in zebrafish. Methods Enzymol 525:159-89 |
| Parikh, Chirag R; Dahl, Neera K; Chapman, Arlene B et al. (2012) Evaluation of urine biomarkers of kidney injury in polycystic kidney disease. Kidney Int 81:784-90 |
| Kuo, Ivana Y; Ehrlich, Barbara E (2012) Ion channels in renal disease. Chem Rev 112:6353-72 |
| Yoshiba, Satoko; Shiratori, Hidetaka; Kuo, Ivana Y et al. (2012) Cilia at the node of mouse embryos sense fluid flow for left-right determination via Pkd2. Science 338:226-31 |
| ?eli?, Andjelka S; Petri, Edward T; Benbow, Jennifer et al. (2012) Calcium-induced conformational changes in C-terminal tail of polycystin-2 are necessary for channel gating. J Biol Chem 287:17232-40 |
| Takiar, Vinita; Mistry, Kavita; Carmosino, Monica et al. (2012) VIP17/MAL expression modulates epithelial cyst formation and ciliogenesis. Am J Physiol Cell Physiol 303:C862-71 |
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