title: Polycystin-1, the gene product of PKD1, induces resistance to apoptosis and spontaneous tubulogenesis in MDCK cells.
abstract: The major form of autosomal dominant polycystic kidney disease (ADPKD) results from mutation of a gene (PKD1) of unknown function that is essential for the later stages of renal tubular differentiation. In this report, we describe a novel cell culture system for studying how PKD1 regulates this process. We show that expression of human PKD1 in MDCK cells slows their growth and protects them from programmed cell death. MDCK cells expressing PKD1 also spontaneously form branching tubules while control cells form simple cysts. Increased cell proliferation and apoptosis have been implicated in the pathogenesis of cystic diseases. Our study suggests that PKD1 may function to regulate both pathways, allowing cells to enter a differentiation pathway that results in tubule formation.
authors: Department of Medicine Division of Nephrology Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
authors: A
authors: A
authors: Boletta
authors: F
authors: F
authors: Qian
authors: L F
authors: LF
authors: Onuchic
authors: A K
authors: AK
authors: Bhunia
authors: B
authors: B
authors: Phakdeekitcharoen
authors: K
authors: K
authors: Hanaoka
authors: W
authors: W
authors: Guggino
authors: L
authors: L
authors: Monaco
authors: G G
authors: GG
authors: Germino
mesh_terms: D000818:Animals; D017209:Apoptosis; D002455:Cell Division; D002460:Cell Line; D003094:Collagen / Q000378:metabolism; D004285:Dogs; D006801:Humans; D007684:Kidney Tubules / Q000166:cytology* / Q000378:metabolism; D008565:Membrane Proteins / Q000378:metabolism; D010641:Phenotype; D016891:Polycystic Kidney, Autosomal Dominant / Q000235:genetics / Q000473:pathology; D011506:Proteins / Q000235:genetics / Q000378:metabolism*; D012333:RNA, Messenger / Q000032:analysis / Q000235:genetics; D011994:Recombinant Proteins / Q000235:genetics / Q000378:metabolism; D050396:TRPP Cation Channels
chemical_list: D003094:Collagen; D008565:Membrane Proteins; D011506:Proteins; D012333:RNA, Messenger; D011994:Recombinant Proteins; D050396:TRPP Cation Channels