Beschreibung:
This volume focuses on methods applied to autosomal dominant polycystic kidney disease (ADPKD), a common human genetic disease. ADPKD is caused by abnormal cilia formation or function. This proposed book will cover the state-of-the-art methods ranging from molecular biology, biochemistry, electrophysiology, to tools in model animal studies.
1. Biochemical Analysis of the Polycystin-1 Complexity Generated by Proteolytic Cleavage at the G Protein-Coupled Receptor Proteolysis Site. 2. Structural Determination of the Polycystin-2 Channel by Electron Cryo-Microscopy. 3. Recording Ion Channels in Cilia Membranes. 4. Electrophysiological Recording of a Gain-of-Function Polycystin-2 Channel with a Two-Electrode Voltage Clamp. 5. Functional Studies of PKD2 and PKD2L1 through Opening the Hydrophobic Activation Gate. 6. Analyzing the GPCR Function of Polycystin-1. 7. Methods to Study the Vasculature in ADPKD. 8. Energy Metabolism, Metabolic Sensors, and Nutritional Interventions in Polycystic Kidney Disease. 9. "Kidney in a Dish" Organoids for PKD. 10. Rodent Autosomal Dominant Polycystic Kidney Disease Models. 11. Using C. elegans as a Model in PKD. 12. Approaches to Studying Polycystic Kidney Disease in Zebrafish. 13. Investigation of DNA Methylation in Autosomal Dominant Polycystic Kidney Disease. 14. Molecular Diagnosis of Autosomal Dominant Polycystic Kidney Disease.