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| 1 | Role of calcium in polycystic kidney disease:From signaling to pathology显示文摘Autosomal dominant polycystic kidney disease(ADPKD) is the most common inherited monogenic kidney disease. Characterized by the development and growth of cysts that cause progressive kidney enlargement, it ultimately leads to end-stage renal disease. Approximately 85% of ADPKD cases are caused by mutations in the PKD1 gene, while mutations in the PKD2 gene account for the remaining 15% of cases. The PKD1 gene encodes for polycystin-1(PC1), a large multi-functional membrane receptor protein able to regulate ion channel complexes, whereas polycystin-2(PC2), encoded by the PKD2 gene, is an integral membrane protein that functions as a calcium-permeable cation channel, located mainly in the endoplasmic reticulum(ER). In the primary cilia of the epithelial cells, PC1 interacts with PC2 to form a polycystin complex that acts as a mechanosensor, regulating signaling pathways involved in the differentiation of kidney tubular epithelial cells. Despite progress in understanding the function of these proteins, the molecular mechanisms associated with the pathogenesis of ADPKD remain unclear. In this review we discuss how an imbalance between functional PC1 and PC2 proteins may disrupt calcium channel activities in the cilium, plasma membrane and ER, thereby altering intracellular calcium signaling and leading to the aberrant cell proliferation and apoptosis associated with the development and growth of renal cysts. Research in this field could lead to the discovery of new molecules able to rebalance intracellular calcium, thereby normalizing cell proliferation and reducing kidney cyst progression. | Alessandra Mangolini Lucia de Stephanis Gianluca Aguiari | 2016 | World Journal of Nephrology2016，5，1： | 5 |
| 2 | Radial structure of a low frequency atmospheric pressure glow discharge in helium显示文摘 | Mangolini L Orlov K Kortshagen U | 2002 | Applied Physics Letters2002，80，10： | 1 |
| 3 | Radial structure of a low-frequency atmospheric-pressure glow discharge in helium显示文摘 | Mangolini L Orlov K Kortshagen U | | 0，，10： | 1 |
| 4 | Reactivity of triphenyl phosphorothionate in lubricant oil solution显示文摘 | Mangolini F Rossi A Spencer N I) | 2009 | TribologT Letters2009，42，35： | 1 |
| 5 | Fusion of Satellite Images of Different Spatial Resolutions: Assessing the Quality of Resulting Images显示文摘 | Wald L Ranchin T Mangolini M | 1997 | PE&RS1997，63，3： | 1 |
| 6 | Pulse plating of sacrificial Mn-Cu alloys from sulphate bath显示文摘 | Mangolini F Cavallotti P L | 2007 | Transactions of the institute of metal finishing2007，85，1： | 1 |
| 7 | Radial structure of a low-frequency atmospheric-pressure glow discharge in helium显示文摘 | Mangolini L Orlov K Kortshagen U | 2002 | App Phys Lett2002，80，10： | 1 |
| 8 | Mitochondrial dysfunction and death in motor neurons exposed to the glutathione depleting agent ethacrynic acid显示文摘 | Rizzardini M Lupi M Mangolini A | 2003 | J Neurol Sci2003，207，： | 1 |
| 9 | Fusion of Satellite Images of Different Spatial Resolutions; Assessing the Quality of Resulting Images显示文摘 | Wald L Ranehin T Mangolini M | 1997 | Photogram metric Engineering & Remote Sensing1997，63，6： | 1 |
| 10 | Fusion of satellite images of different spatial resolutions:Assessing the quality of resulting images显示文摘 | Wald L Ranchin T Mangolini M | 1997 | Photogrammetric Engineering & Remote Sensing1997，63，6： | 1 |
| 11 | Silicon nanocrystals with ensemble quantum yields excee- ding 60% 显示文摘 | JURBERGS D ROGOJINA E MANGOLINI L | 2006 | Applied Physics Letters2006，88，23： | 1 |
| 12 | Effect of currentlimitation through the dielectric in atmospheric pressure glows in helium显示文摘 | Mangolini L Anderson C Heberlein J Kortshagen | 2004 | J Phys D: Appl Phys2004，37，： | 1 |
| 13 | Low levels of ALS-linked Cu/Zn superoxide dismutase increase the production of reactive oxygen species and cause mitochondrial damage and death in motor neuron-like cells显示文摘 | RIZZARDINI M MANGOLINI A LUPI M | 2005 | J Neurol Sci2005，232，12： | 1 |
| 14 | Fusion of satellite images of different spatial resolutions:Assessing the quality of resulting images显示文摘 | Wald L Ranchin T Mangolini M | 1997 | Photogrammetric Engineering and Remote Sensing1997，63，6： | 1 |
| 15 | Fusion of Satellite Images of Different Spatial Resolutions:Assessing the Quality of Resulting Images显示文摘 | Wald L Ranchin T Mangolini | 1997 | Photogrammetric Engineering and Remote Sensing1997，63，： | 1 |
| 16 | Fusion of satellite images of different spatial resolutions : Assessing the quality of resulting images 显示文摘 | WALD L RANCHIN T MANGOLINI M | 1997 | Photogrammetry Eng Remote Sensing1997，63，6： | 1 |
| 17 | Effects of cur- rent limitation through the dielectric in atmospheric pressure glows in helium显示文摘 | Mangolini L Anderson C Heberlein J | 2004 | Journal of Physics D: Applied Physics2004，37，1： | 1 |
| 18 | High-yield plasma synthesis of luminescent silicon nanoerystals显示文摘 | Mangolini L Thimsen E Kortshagen U | 2005 | Nano Lett2005，5，4： | 1 |
| 19 | Radial structure of a low-frequency atmospheric-pressure glow discharge in helium显示文摘 | Mangolini L Orlov K Kortshagen U | | 0，，10： | 1 |
| 20 | Radial Structure of a Low-Frequency Atmospheric-Pressure Glow Discharge in Helium显示文摘 | Mangolini L Orlov K Kortschagen U | 2002 | Applied Physics Letters2002，80，10： | 1 |
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