This volume focuses on the investigatory methods applied to autosomal dominant polycystic kidney disease (ADPKD), one of the most common human genetic diseases. ADPKD is caused by mutations in PKD1 and TRPP2, two integral membrane proteins that function as receptor/ion channels in primary cilia of tubular epithelial cells. Thus, ADPKD belongs to ciliopathies, a group of disorders 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.

Key Features

  • Explores the role of cilia in polycystic kidney disease
  • Focuses on myriad state-of-the-art methods and techniques
  • Reviews specific mutations integral to this autosomal genetic disease
  • Includes discussions of model systems

chapter 3|18 pages

Recording Ion Channels in Cilia Membranes

ByLeo C.T. Ng, Amitabha Mukhopadhyay, Thuy N. Vien, Paul G. DeCaen

chapter 4|17 pages

Electrophysiological Recording of a Gain-of-Function Polycystin-2 Channel with a Two-Electrode Voltage Clamp

ByCourtney Ng, Zhifei Wang, Bin Li, Yong Yu

chapter 5|16 pages

Functional Studies of PKD2 and PKD2L1 through Opening the Hydrophobic Activation Gate

ByWang Zheng, Lingyun Wang, Jingfeng Tang, Ji-Bin Peng, Xing-Zhen Chen

chapter 6|24 pages

Analyzing the GPCR Function of Polycystin-1

ByStephen C. Parnell, Robin L. Maser, Brenda S. Magenheimer, James P. Calvet

chapter 7|34 pages

Methods to Study the Vasculature in ADPKD

ByPatricia Outeda, Terry Watnick

chapter 8|15 pages

Energy Metabolism, Metabolic Sensors, and Nutritional Interventions in Polycystic Kidney Disease

BySonu Kashyap, Eduardo Nunes Chini

chapter 9|18 pages

“Kidney in a Dish” Organoids for PKD

ByNelly M. Cruz, Benjamin S. Freedman

chapter 10|51 pages

Rodent Autosomal Dominant Polycystic Kidney Disease Models

BySara J. Holditch, Raphael A. Nemenoff, Katharina Hopp

chapter 11|20 pages

Using C. elegans as a Model in PKD

ByJuan Wang, Maureen Barr

chapter 12|26 pages

Approaches to Studying Polycystic Kidney Disease in Zebrafish

ByJingyu Li, Ying Cao

chapter 13|16 pages

Investigation of DNA Methylation in Autosomal Dominant Polycystic Kidney Disease

ByEwud Agborbesong, Xiaogang Li

chapter 14|21 pages

Molecular Diagnosis of Autosomal Dominant Polycystic Kidney Disease

ByMatthew Lanktree, Amirreza Haghighi, Xueweng Song, York Pei