Advances in Experimental Medicine and Biology

Cystogenesis

Editors: Park, Jong Hoon, Ahn, Curie (Eds.)

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  • 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
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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.

Table of contents (9 chapters)

Table of contents (9 chapters)

Buy this book

eBook $139.00
price for USA in USD
  • ISBN 978-981-10-2041-4
  • Digitally watermarked, DRM-free
  • Included format: PDF, EPUB
  • Immediate eBook download after purchase and usable on all devices
  • Bulk discounts available
Hardcover $179.99
price for USA in USD
Softcover $179.99
price for USA in USD
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Bibliographic Information

Bibliographic Information
Book Title
Cystogenesis
Editors
  • Jong Hoon Park
  • Curie Ahn
Series Title
Advances in Experimental Medicine and Biology
Series Volume
933
Copyright
2016
Publisher
Springer Singapore
Copyright Holder
Springer Science+Business Media Singapore
eBook ISBN
978-981-10-2041-4
DOI
10.1007/978-981-10-2041-4
Hardcover ISBN
978-981-10-2040-7
Softcover ISBN
978-981-10-9511-5
Series ISSN
0065-2598
Edition Number
1
Number of Pages
IX, 126
Topics