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Cystogenesis (Hardcover, 1st ed. 2016)
Loot Price: R3,530
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Cystogenesis (Hardcover, 1st ed. 2016)
Series: Advances in Experimental Medicine and Biology, 933
Expected to ship within 10 - 15 working days
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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.
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