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Cystogenesis

  • Book
  • © 2016

Overview

Editors:
  1. Jong Hoon Park

    1. Department of Life systems, Sookmyung Women’s University, Seoul, Korea (Republic of)

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  2. Curie Ahn

    1. Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea (Republic of)

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    You can also search for this editor in PubMed   Google Scholar

  • Presents key challenges in the study of Autosomal dominant polycystic kidney disease (ADPKD)
  • Covers basic cellular mechanisms and clinical trials in ADPKD research
  • Opens the door for the development of biomarkers and therapeutic targets for ADPKD

Part of the book series: Advances in Experimental Medicine and Biology (AEMB, volume 933)

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Table of contents (9 chapters)

  1. Front Matter

    Pages i-ix
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  2. ADPKD Overview

    1. Front Matter

      Pages 1-1
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    2. Recent Trends in ADPKD Research

      • Yu Bin Shin, Jong Hoon Park
      Pages 3-11

    3. Genetic Mechanisms of ADPKD

      • Do Yeon Kim, Jong Hoon Park
      Pages 13-22

  3. Cystogenesis Mechanisms

    1. Front Matter

      Pages 23-23
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    2. Cell Proliferation and Apoptosis in ADPKD

      • Eun Ji Lee
      Pages 25-34

    3. Inflammation and Fibrosis in ADPKD

      • Hyowon Mun, Jong Hoon Park
      Pages 35-44

    4. Functional Study of the Primary Cilia in ADPKD

      • Je Yeong Ko
      Pages 45-57

    5. Epigenetic Regulation in Cystogenesis

      • Yu Mi Woo
      Pages 59-68

  4. Therapeutic Approaches and Diagnostic Markers for ADPKD

    1. Front Matter

      Pages 69-69
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    2. Validation of Effective Therapeutic Targets for ADPKD Using Animal Models

      • Yu Mi Woo, Je Yeong Ko, Eun Ji Lee
      Pages 71-84

    3. Diagnostic Evaluation as a Biomarker in Patients with ADPKD

      • Hayne Cho Park, Curie Ahn
      Pages 85-103

    4. Clinical Trials and a View Toward the Future of ADPKD

      • Hyunsuk Kim, Young-Hwan Hwang
      Pages 105-121

  5. Back Matter

    Pages 123-126
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Keywords

About this book

Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a highly prevalent hereditary renal disorder in which fluid-filled cysts are appeared in both kidneys. Main causative genes of ADPKD are PKD1 and PKD2, encoding for polycystin-1 (PC1) and polycystin-2 (PC2) respectively. Those proteins are localized on primary cilia and function as mechanosensor in response to the fluid flow, translating mechanistic stimuli into calcium signaling. With mutations either of PKD1 or PKD2, hyper-activated renal tubular epithelial cell proliferation is observed, followed by disrupted calcium homeostasis and aberrant intracellular cyclic AMP (cAMP) accumulation. Increased cell proliferation with fluid secretion leads to the development of thousands of epithelial-lined, fluid-filled cysts in kidneys. It is also accompanied by interstitial inflammation, fibrosis, and finally reaching end-stage renal disease (ESRD). In human ADPKD, the age at which renal failure typically occurs is later in life, however no specific targeted medications are available to cure ADPKD. Recently, potential therapeutic targets or surrogate diagnostic biomarkers for ADPKD are proposed with the advances in the understanding of ADPKD pathogenesis, and some of them were attempted for clinical trials. Herein, we will summarize genetic and epi-genetic molecular mechanisms in ADPKD progression, and overview the currently available biomarkers or potential therapeutic reagents suggested.

Editors and Affiliations

  • Department of Life systems, Sookmyung Women’s University, Seoul, Korea (Republic of)

    Jong Hoon Park

  • Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea (Republic of)

    Curie Ahn

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