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Titlebook: Cystogenesis; Jong Hoon Park,Curie Ahn Book 2016 The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Natu

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Functional Study of the Primary Cilia in ADPKD (PKD), suggesting that the malformation or absence of primary cilia is a driving force of the onset of PKD. Therefore, in this chapter, the renal cystogenesis mechanism induced by cilia defects and pathogenic ciliary proteins associated with PKD development will be described.
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Diagnostic Evaluation as a Biomarker in Patients with ADPKDter from the treatment, and (3) they should be easy to evaluate short-term outcome after treatment, which will demonstrate hard outcome. Herein, we will discuss currently available surrogate biomarkers including the volume of total kidney and urinary molecular markers.
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Validation of Effective Therapeutic Targets for ADPKD Using Animal Modelsches using PKD animal models would shed light on the development of potential therapeutic strategies for PKD. Here, we provide an update on the current evidence obtained by the in vivo evaluation of PKD therapeutic candidates and discuss the effect of therapeutic targets.
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https://doi.org/10.1007/978-3-642-91395-2 pathways, such as the negative growth regulation, G protein activation, and canonical and non-canonical Wnt pathways. According to the “second hit” model, an additional somatic mutation results in the expansion of cyst growth. In this chapter we discuss the genetic mechanisms and signaling pathways involved in ADPKD.
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0065-2598 rials in ADPKD research.Opens the door for the development oAutosomal 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)
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G. H. Simmons M.D.,M. Fislibein M.D.ches using PKD animal models would shed light on the development of potential therapeutic strategies for PKD. Here, we provide an update on the current evidence obtained by the in vivo evaluation of PKD therapeutic candidates and discuss the effect of therapeutic targets.
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