书目名称 | Cystogenesis | 编辑 | Jong Hoon Park,Curie Ahn | 视频video | | 概述 | 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 o | 丛书名称 | Advances in Experimental Medicine and Biology | 图书封面 |  | 描述 | 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 | 出版日期 | Book 2016 | 关键词 | Autosomal Dominant Polycystic Kidney Disease (ADPKD); Cyst; End-stage Renal Disease (ESRD); Genetic mec | 版次 | 1 | doi | https://doi.org/10.1007/978-981-10-2041-4 | isbn_softcover | 978-981-10-9511-5 | isbn_ebook | 978-981-10-2041-4Series ISSN 0065-2598 Series E-ISSN 2214-8019 | issn_series | 0065-2598 | copyright | The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Singapor |
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