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Fibrocystin/polyductin modulates renal tubular formation by regulating polycystin-2 expression and function.

Kim I, Fu Y, Hui K, Moeckel G, Mai W, Li C, Liang D, Zhao P, Ma J, Chen XZ, George AL Jr, Coffey RJ, Feng ZP, Wu G.

J Am Soc Nephrol. 2008 Mar;19(3):455-68. doi: 10.1681/ASN.2007070770. Epub 2008 Jan 30.


Fibrocystin/polyductin, found in the same protein complex with polycystin-2, regulates calcium responses in kidney epithelia.

Wang S, Zhang J, Nauli SM, Li X, Starremans PG, Luo Y, Roberts KA, Zhou J.

Mol Cell Biol. 2007 Apr;27(8):3241-52. Epub 2007 Feb 5.


Molecular basis of autosomal recessive polycystic kidney disease (ARPKD).

Al-Bhalal L, Akhtar M.

Adv Anat Pathol. 2008 Jan;15(1):54-8. Review.


Polyductin, the PKHD1 gene product, comprises isoforms expressed in plasma membrane, primary cilium, and cytoplasm.

Menezes LF, Cai Y, Nagasawa Y, Silva AM, Watkins ML, Da Silva AM, Somlo S, Guay-Woodford LM, Germino GG, Onuchic LF.

Kidney Int. 2004 Oct;66(4):1345-55.


Inhibition of Pkhd1 impairs tubulomorphogenesis of cultured IMCD cells.

Mai W, Chen D, Ding T, Kim I, Park S, Cho SY, Chu JS, Liang D, Wang N, Wu D, Li S, Zhao P, Zent R, Wu G.

Mol Biol Cell. 2005 Sep;16(9):4398-409. Epub 2005 Jun 22.


PKHD1 protein encoded by the gene for autosomal recessive polycystic kidney disease associates with basal bodies and primary cilia in renal epithelial cells.

Zhang MZ, Mai W, Li C, Cho SY, Hao C, Moeckel G, Zhao R, Kim I, Wang J, Xiong H, Wang H, Sato Y, Wu Y, Nakanuma Y, Lilova M, Pei Y, Harris RC, Li S, Coffey RJ, Sun L, Wu D, Chen XZ, Breyer MD, Zhao ZJ, McKanna JA, Wu G.

Proc Natl Acad Sci U S A. 2004 Feb 24;101(8):2311-6.


Cellular and subcellular localization of the ARPKD protein; fibrocystin is expressed on primary cilia.

Ward CJ, Yuan D, Masyuk TV, Wang X, Punyashthiti R, Whelan S, Bacallao R, Torra R, LaRusso NF, Torres VE, Harris PC.

Hum Mol Genet. 2003 Oct 15;12(20):2703-10. Epub 2003 Aug 12.


Polycystic kidney disease protein fibrocystin localizes to the mitotic spindle and regulates spindle bipolarity.

Zhang J, Wu M, Wang S, Shah JV, Wilson PD, Zhou J.

Hum Mol Genet. 2010 Sep 1;19(17):3306-19. doi: 10.1093/hmg/ddq233. Epub 2010 Jun 16.


Molecular and cellular pathogenesis of autosomal recessive polycystic kidney disease.

Menezes LF, Onuchic LF.

Braz J Med Biol Res. 2006 Dec;39(12):1537-48. Review.


A mouse model of autosomal recessive polycystic kidney disease with biliary duct and proximal tubule dilatation.

Woollard JR, Punyashtiti R, Richardson S, Masyuk TV, Whelan S, Huang BQ, Lager DJ, vanDeursen J, Torres VE, Gattone VH, LaRusso NF, Harris PC, Ward CJ.

Kidney Int. 2007 Aug;72(3):328-36. Epub 2007 May 23.


Regulation of cell proliferation and apoptosis through fibrocystin-prosaposin interaction.

Sun L, Wang S, Hu C, Zhang X.

Arch Biochem Biophys. 2010 Oct 15;502(2):130-6. doi: 10.1016/ Epub 2010 Aug 13.


Biliary and pancreatic dysgenesis in mice harboring a mutation in Pkhd1.

Gallagher AR, Esquivel EL, Briere TS, Tian X, Mitobe M, Menezes LF, Markowitz GS, Jain D, Onuchic LF, Somlo S.

Am J Pathol. 2008 Feb;172(2):417-29. doi: 10.2353/ajpath.2008.070381. Epub 2008 Jan 17.


Defects in cholangiocyte fibrocystin expression and ciliary structure in the PCK rat.

Masyuk TV, Huang BQ, Ward CJ, Masyuk AI, Yuan D, Splinter PL, Punyashthiti R, Ritman EL, Torres VE, Harris PC, LaRusso NF.

Gastroenterology. 2003 Nov;125(5):1303-10.


Kinesin-2 mediates physical and functional interactions between polycystin-2 and fibrocystin.

Wu Y, Dai XQ, Li Q, Chen CX, Mai W, Hussain Z, Long W, Montalbetti N, Li G, Glynne R, Wang S, Cantiello HF, Wu G, Chen XZ.

Hum Mol Genet. 2006 Nov 15;15(22):3280-92. Epub 2006 Sep 28.


The cytoplasmic tail of FPC antagonizes the full-length protein in the regulation of mTOR pathway.

Wang S, Wu M, Yao G, Zhang J, Zhou J.

PLoS One. 2014 May 22;9(5):e95630. doi: 10.1371/journal.pone.0095630. eCollection 2014.


A novel model of autosomal recessive polycystic kidney questions the role of the fibrocystin C-terminus in disease mechanism.

Outeda P, Menezes L, Hartung EA, Bridges S, Zhou F, Zhu X, Xu H, Huang Q, Yao Q, Qian F, Germino GG, Watnick T.

Kidney Int. 2017 Nov;92(5):1130-1144. doi: 10.1016/j.kint.2017.04.027. Epub 2017 Jul 18.


Polycystic kidney disease--the ciliary connection.

Ong AC, Wheatley DN.

Lancet. 2003 Mar 1;361(9359):774-6.


[From gene to disease; PKHD1 and recessive polycystic kidney disease].

Peters DJ, Losekoot M, de Die-Smulders CE, Stevens-Baldewijns M, Breuning MH.

Ned Tijdschr Geneeskd. 2005 Feb 26;149(9):463-6. Review. Dutch.


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