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Autosomal recessive polycystic kidney disease: Difference between revisions

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==Genetics==
The cause of ARPKD is linked to mutations in the PKHD1 gene.<ref name="kidn">{{Cite web|title = Polycystic kidney disease|url = http://ghr.nlm.nih.gov/condition/polycystic-kidney-disease|website = Genetics Home Reference|accessdate = 2015-07-28}}</ref> PKHD1 gene codes forfibrocystin. Fibrocystin is found in the epithelial cells of both the renal tubule and the bile ducts; deficiency leads to the characteristic polycystic dilation of both structures<ref>Kim I, Fu Y, Hui K, et al. Fibrocystin/polyductin modulates renal tubular formation by regulating polycystin-2 expression and function. J Am Soc Nephrol. 2008;19(3):455-468. doi:10.1681/ASN.2007070770</ref>
 
[[Image:Von Meyenburg complex low mag.jpg|150px|thumb|left|[[Micrograph]] of [[von Meyenburg complex]].]]