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Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium
Published on: September 1, 2015
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Functional Study of the Primary Cilia in ADPKD
1Molecular Medicine Laboratory, Department of Life systems, Sookmyung Women's University, Cheongpa-ro 47-gil 100, Yongsan-gu, Seoul, 04310, South Korea. jeyeong@sookmyung.ac.kr.
Advances in Experimental Medicine and Biology
|October 13, 2016
Summary
Primary cilia act as cellular antennae, and their defects, particularly involving polycystin proteins, are linked to polycystic kidney disease (PKD) development. This chapter details how cilia dysfunction drives renal cystogenesis in PKD.
Area of Science:
- Cell Biology
- Nephrology
- Genetics
Background:
- Primary cilia are microtubule-based cellular antennae crucial for signal transduction.
- These organelles are vital in kidney tubules, sensing fluidic stress.
- Defects in primary cilia are increasingly implicated in kidney diseases.
Purpose of the Study:
- To elucidate the mechanism of renal cystogenesis driven by primary cilia defects.
- To describe pathogenic ciliary proteins associated with polycystic kidney disease (PKD).
Main Methods:
- Review of recent studies on ciliary protein function in renal tubules.
- Analysis of signaling pathways localized to primary cilia.
- Investigation of the role of polycystin-1 (PC1), polycystin-2 (PC2), and intraflagellar transport (IFT) proteins.
Main Results:
- Proteins for Wnt, PDGFRα, Hh, and mechanosignaling pathways localize to primary cilia.
- Disruption of PC1, PC2, and IFT proteins is linked to PKD development.
- Primary cilia malformation or absence is a key driver of PKD onset.
Conclusions:
- Primary cilia defects are central to the pathogenesis of polycystic kidney disease.
- Understanding ciliary protein function is critical for targeting PKD therapies.
- This chapter provides insights into cilia-mediated renal cystogenesis.
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