The Gretchen question in autosomal-dominant polycystic kidney disease research

Luft, Friedrich C.

doi:10.1007/s00109-011-0734-6

Cited by 2 publications

(2 citation statements)

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“…Once in the interstitium the cysts continue to slowly expand over many decades (Grantham et al, 2011). Kidney failure therefore occurs after a long latent period, usually by the 5th decade of life (Baert, 1978), when a sufficient number of cysts (possibly > 1000) (Luft, 2011) have collectively grown to disrupt normal kidney architecture and function. In contrast, ARPKD is a less frequent, childhood disease (1:20 000 live births) (Sweeney and Avner, 2006).…”

Section: Overview Of Pkdmentioning

confidence: 99%

“…Nevertheless, significant insights have been gained from this limited information to support that extracellular nucleotide signaling is worthy of further pursuit in PKD. In fact, it has been hypothesized that this approach might act synergistically with vasopressin receptor antagonism (Buchholz et al, 2011; Luft, 2011). Extracellular nucleotides and metabolites can be classified according to the base from which they are derived and the number of associated number of phosphate groups (adenosine-derived; ATP, ADP; AMP; guanosine-derived: GTP, GDP, GMP; cytidine-derived: CTP, CDP, CMP; 5-methyluridine- and uridine-derived).…”

Section: Introductionmentioning

confidence: 99%

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Role of extracellular ATP and P2 receptor signaling in regulating renal cyst growth and interstitial inflammation in polycystic kidney disease

Rangan¹

2013

Front. Physiol.

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Polycystic kidney diseases (PKD) are a group of inherited ciliopathies in which the formation and growth of multiple cysts derived from the distal nephron and collecting duct leads to the disruption of normal kidney architecture, chronic interstitial inflammation/fibrosis and hypertension. Kidney failure is the most life-threatening complication of PKD, and is the consequence of cyst expansion, renal interstitial disease and loss of normal kidney tissue. Over the last decade, accumulating evidence suggests that the autocrine and paracrine effects of ATP (through its receptor family P2X and P2Y), could be detrimental for the progression of PKD. (2009). In vitro, ATP-P2 signaling promotes cystic epithelial cell proliferation, chloride-driven fluid secretion and apoptosis. Furthermore, dysfunction of the polycystin signal transduction pathways promotes the secretagogue activity of extracellular ATP by activating a calcium-activated chloride channel via purinergic receptors. Finally, ATP is a danger signal and could potentially contribute to interstitial inflammation associated with PKD. These data suggest that ATP-P2 signaling worsens the progression of cyst enlargement and interstitial inflammation in PKD.

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