title: Altered extracellular matrix component gene expression in murine polycystic kidney.
abstract: The DBA/2FG-pcy mouse has a form of slowly progressive kidney disease that appears similar in many respects to that seen in the autosomal dominant form of human polycystic kidney disease. This study was designed to assess how the expression of extracellular matrix component genes is regulated in a model of murine polycystic kidney disease and control DBA/2 mice at 8, 16, and 30 weeks of age. The mRNA levels encoding for collagen IV, the B1 and B2 chains of laminin, heparan sulfate proteoglycan, fibronectin, and collagens I and III increased with the progression of cystic lesions in the kidney of DBA/2FG-pcy mice. At 30 weeks of age, mRNA levels for collagen IV, laminin B1 and B2, heparan sulfate proteoglycan, fibronectin, and collagens I and III were increased 8.1-fold, 7.0-fold, 7.0-fold, 9.8-fold, 7.0-fold, 5.5-fold, and 5.4-fold, respectively, compared to those of control DBA/2 mice. An immunofluorescence study revealed the irregular staining for collagen IV, laminin, heparan sulfate proteoglycan, and collagens I and III around the cysts. These data suggest that changes in the expression of basement membrane components and interstitial collagens are associated with the development of polycystic kidney disease.
authors.affiliation: Department of Medicine, Juntendo University School of Medicine, Tokyo, Japan.
authors.forename: I
authors.initials: I
authors.lastname: Ebihara
authors.forename: T
authors.initials: T
authors.lastname: Nakamura
authors.forename: T
authors.initials: T
authors.lastname: Takahashi
authors.forename: M
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Y
authors.initials: Y
authors.lastname: Tomino
authors.forename: S
authors.initials: S
authors.lastname: Nagao
authors.forename: H
authors.initials: H
authors.lastname: Takahashi
authors.forename: H
authors.initials: H
authors.lastname: Koide
mesh_terms: D000818:Animals; D015152:Blotting, Northern; D003094:Collagen / Q000235:genetics; D016326:Extracellular Matrix Proteins / Q000235:genetics* / Q000378:metabolism; D005353:Fibronectins / Q000235:genetics; D005455:Fluorescent Antibody Technique; D005786:Gene Expression Regulation; D006493:Heparin / Q000031:analogs & derivatives / Q000235:genetics; D007668:Kidney / Q000378:metabolism; D007797:Laminin / Q000235:genetics; D051379:Mice; D008811:Mice, Inbred DBA; D007690:Polycystic Kidney Diseases / Q000235:genetics*; D011506:Proteins / Q000235:genetics* / Q000378:metabolism; D011509:Proteoglycans / Q000235:genetics; D012333:RNA, Messenger / Q000032:analysis; D050396:TRPP Cation Channels
chemical_list: D003094:Collagen; D016326:Extracellular Matrix Proteins; D005353:Fibronectins; D006493:Heparin; D007797:Laminin; D011506:Proteins; D011509:Proteoglycans; D012333:RNA, Messenger; D050396:TRPP Cation Channels; C036090:heparin proteoglycan