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Tijdschrift voor Kindergeneeskunde
Gepubliceerd in:

01-04-2006 | Artikelen

De rol van polycystine-1 en polycystine-2 in de pathofysiologie van cystennieren

Auteur: dr. D. J. M. Peters

Gepubliceerd in: Tijdschrift voor Kindergeneeskunde | Uitgave 2/2006

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Summary

In recent years, our knowledge on the pathophysiology of polycystic kidney disease expanded dramatically. An increasing number of genes mutated in renal cystic diseases in humans or rodents have been identified and most of them encode proteins that localise in the primary cilium, a mechanosensor that regulates intracellular [Ca2+]i levels. Polycystin-1 and polycystin-2, the proteins encoded by the genes mutated in patients with autosomal dominant polycystic kidney disease (adpkd), seem to play a crucial role in this regulation. The polycystins belong to a novel subclass of transient receptor potential (trp) channels, the transient receptor potential polycystins (trpp's). Increased knowledge on altered signaling in polycystic kidney disease and the availability of a variety of animal models are now providing the opportunities to test therapeutic interventions.
vorige artikel De ziekte van Dent
volgende artikel Aquaporines in de nier en hun rol in nefrogene diabetes insipidus
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Metagegevens
Titel
De rol van polycystine-1 en polycystine-2 in de pathofysiologie van cystennieren
Auteur
dr. D. J. M. Peters
Publicatiedatum
01-04-2006
Uitgeverij
Bohn Stafleu van Loghum
Gepubliceerd in
Tijdschrift voor Kindergeneeskunde / Uitgave 2/2006
Print ISSN: 0376-7442
Elektronisch ISSN: 1875-6840
DOI
https://doi.org/10.1007/BF03061609

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