interesting Polycystic Kidney Disease research

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interesting Polycystic Kidney Disease research

Post by bigbuzzard »

This was published in Nature Genetics earlier this month. ... t%29Nature

Very early days but pretty amazing if this develops!


Initiation of cyst formation in autosomal dominant polycystic kidney disease (ADPKD) occurs when kidney tubule cells are rendered null for either PKD1 or PKD2 by somatic ‘second hit’ mutations. Subsequent cyst progression remodels the organ through changes in tubule cell shape, proliferation and secretion. The kidney develops inflammation and fibrosis. We constructed a mouse model in which adult inactivation of either Pkd gene can be followed by reactivation of the gene at a later time. Using this model, we show that re-expression of Pkd genes in cystic kidneys results in rapid reversal of ADPKD. Cyst cell proliferation is reduced, autophagy is activated and cystic tubules with expanded lumina lined by squamoid cells revert to normal lumina lined by cuboidal cells. Increases in inflammation, extracellular matrix deposition and myofibroblast activation are reversed, and the kidneys become smaller. We conclude that phenotypic features of ADPKD are reversible and that the kidney has an unexpected capacity for plasticity controlled at least in part by ADPKD gene function.
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Re: interesting Polycystic Kidney Disease research

Post by Thumps »

Wow, that does sound promising!

I remember reading a research paper some years ago about how even calling the disease PKD seems a bit of a misnomer given that it is so frequently accompanied my Polycystic Liver, diverticulitis and aneurysm, which made the folks feel like it was more of a collagen disorder?

If this were to be the case then perhaps this treatment will help with those adverse effects also.
PKD/PLD diagnosed 1994, CKD stage 5 Oct 2007, Living Donor Kidney Transplant Dec 2008, still going strong!
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