Disruption of polycystin-1 function interferes with branching morphogenesis of the ureteric bud in developing mouse kidneys

Katalin Polgar; Christopher R. Burrow; Deborah P. Hyink; Hilda Fernandez; Katie Thornton; Xiaohong Li; G. Luca Gusella; Patricia D. Wilson

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Disruption of polycystin-1 function interferes with branching morphogenesis of the ureteric bud in developing mouse kidneys

机译：Polycystin-1功能的破坏会干扰发育中的小鼠肾脏中输尿管芽的分支形态发生

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Thepolycystickidneydisease(emPKD1/em)gene-encodedprotein,polycystin-1,isdevelopmentallyregulated,withhighestexpressionlevelsseeninnormaldevelopingkidneys,whereitisdistributedinapunctatepatternatthebasalsurfaceofuretericbudepithelia.OverexpressioninuretericepithelialcellmembranesofaninhibitorypMyr-GFP-PKD1fusionproteinviaaretroviral(VVC)deliverysystemandmicroinjectionintotheuretericbudlumenofembryonicday11mousemetanephrickidneysresultedindisruptedbranchingmorphogenesis.Usingconfocalquantitativeanalysis,significantreductionsweremeasuredinthenumbersofuretericbudbranchpointsandtips,aswellasinthetotaluretericbudlength,volumeandarea,whilesignificantincreaseswereseenasdilationsoftheterminalbranches,wheresignificantincreasesinouterdiameterandvolumesweremeasured.Microinjectionofanactivating5TM-GFP-PKD1fusionproteinhadanoppositeeffectandshowedsignificantincreasesinuretericbudlengthandarea.Thesearethefirststudiestoexperimentallymanipulatepolycystin-1expressionbytransductionintheembryonicmousekidneyandsuggestthatpolycystin-1playsacriticalroleintheregulationofepithelialmorphogenesisduringrenaldevelopment./p/div

机译：Thepolycystickidneydisease（ PKD1 ）基因encodedprotein，多囊蛋白1，isdevelopmentallyregulated，withhighestexpressionlevelsseeninnormaldevelopingkidneys，whereitisdistributedinapunctatepatternatthebasalsurfaceofuretericbudepithelia.OverexpressioninuretericepithelialcellmembranesofaninhibitorypMyr-GFP-PKD1fusionproteinviaaretroviral（VVC）deliverysystemandmicroinjectionintotheuretericbudlumenofembryonicday11mousemetanephrickidneysresultedindisruptedbranchingmorphogenesis.Usingconfocalquantitativeanalysis，significantreductionsweremeasuredinthenumbersofuretericbudbranchpointsandtips，aswellasinthetotaluretericbudlength，volumeandarea，whilesignificantincreaseswereseenasdilationsoftheterminalbranches，wheresignificantincreasesinouterdiameterandvolumesweremeasured.Microinjectionofanactivating5TM-GFP -PKD1融合蛋白具有相反的作用，并显示出明显增加的尿道预算长度和面积。这些是实验性地通过胚胎小鼠转导来操纵多囊藻蛋白-1表达的研究。肾脏，并建议polycystin-1在肾发育过程中对上皮形态发生的调节起关键作用。 展开▼

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《Developmental biology》 |2005年第1期|共页

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Katalin Polgar; Christopher R. Burrow; Deborah P. Hyink; Hilda Fernandez; Katie Thornton; Xiaohong Li; G. Luca Gusella; Patricia D. Wilson;
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1. Disruption of polycystin-1 function interferes with branching morphogenesis of the ureteric bud in developing mouse kidneys. [J] . Polgar K, Burrow CR, Hyink DP, Developmental biology . 2005,第1期

机译：polycystin-1功能的破坏会干扰发育中的小鼠肾脏中输尿管芽的分支形态发生。

2. Protein Kinase X Activates Ureteric Bud Branching Morphogenesis in Developing Mouse Metanephric Kidney [J] . Christopher R. Burrow, Deborah P. Hyink, G. Luca Gusella, Journal of the American Society of Nephrology: JASN . 2005,第12期

机译：蛋白激酶X激活正在发展的小鼠肾肾中的输尿管芽分支形态发生。

3. Protein kinase X activates ureteric bud branching morphogenesis in developing mouse metanephric kidney. [J] . Li X, Hyink DP, Polgar K, Journal of the American Society of Nephrology: JASN . 2005,第12期

机译：蛋白激酶X激活发育中的小鼠后肾的输尿管芽分支形态发生。

4. Dynamic Image-Based Modelling of Kidney Branching Morphogenesis [C] . Srivathsan Adivarahan, Denis Menshykau, Odysse Michos, International conference on computational methods in systems biology . 2013

机译：基于动态图像的肾脏分支形态发生建模

5. Analysis of Ureteric Bud Morphogenesis by Reassociation of Fetal Kidney Cells. [D] . Leclerc, Kevin. 2016

机译：通过胎儿肾脏细胞再结合分析输尿管芽形态。

6. Transcriptional Dysregulation in the Ureteric Bud Causes Multicystic Dysplastic Kidney by Branching Morphogenesis Defect [O] . Qiusha Guo, Piyush Tripathi, Scott R. Manson, -1

机译：输尿管芽中转录异常通过分支形态发生缺陷导致多囊性肾发育异常。

7. Disruption of polycystin-1 function interferes with branching morphogenesis of the ureteric bud in developing mouse kidneys [O] . Polgar Katalin, Burrow Christopher R., Hyink Deborah P., 2005

机译：Polycystin-1功能的破坏会干扰发育中的小鼠肾脏中输尿管芽的分支形态发生

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机译：鼠标和人类输尿管芽有机体和收集管有机体的方法

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机译：诱导肾细胞培养物的微管发生和/或分支形态发生的方法

5. prophylactic or therapeutic treatment method of chronic kidney disease in an individual, prophylactic or therapeutic treatment method of renal insufficiency in an individual, prophylactic or therapeutic treatment method of renal fibrosis in an individual, method to reduce levels, inhibit or reduce the production of profibrotic mediators in an individual at risk of developing or who have chronic kidney disease, renal sufficiency and / or renal fibrosis established, a method for reducing collagen formation or enhancing collagen degradation in an individual's kidneys, peptide vasoactive bowel (piv) or one or more functional fragments of piv, composition for the prophylactic or therapeutic treatment of chronic kidney disease, composition for the prophylactic or therapeutic treatment of renal failure and composition for the prophylactic or therapeutic treatment of renal fibrosis [P] . 外国专利： BRPI0914504A2 . 2016-01-12

机译：个体慢性肾脏病的预防或治疗方法，个体肾功能不全的预防或治疗方法，个体肾纤维化的预防或治疗方法，降低水平，抑制或减少纤维化介质产生的方法在具有发展为慢性病或患有慢性肾脏病，肾功能衰竭和/或肾纤维化的个体中，减少个体肾脏，胶原蛋白血管活性肠（piv）或一个或多个功能片段的胶原蛋白形成或增强胶原蛋白降解的方法静脉注射，用于预防或治疗慢性肾脏疾病的组合物，用于预防或治疗肾衰竭的组合物以及用于预防或治疗肾纤维化的组合物

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Disruption of polycystin-1 function interferes with branching morphogenesis of the ureteric bud in developing mouse kidneys

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