title: Tenc1-deficient mice develop glomerular disease in a strain-specific manner.
abstract: OBJECTIVE
  Tenc1 (also known as tensin2) is an integrin-associated focal adhesion molecule that is broadly expressed in mouse tissues including the liver, muscle, heart and kidney. A mouse strain carrying mutated Tenc1, the ICR-derived glomerulonephritis (ICGN) strain, develops severe nephrotic syndrome.
  
  
  METHODS
  To elucidate the function of Tenc1 in the kidney, Tenc1(ICGN) was introduced into 2 genetic backgrounds, i.e. DBA/2J (D2) and C57BL/6J (B6), strains that are respectively susceptible and resistant to chronic kidney disease.
  
  
  RESULTS
  Biochemical and histological analysis revealed that homozygous Tenc1(ICGN) mice develop nephrotic syndrome on the D2 background (D2GN) but not on the B6 background (B6GN). Initially, abnormal assembly and maturation of glomerular basement membrane (GBM) were observed, and subsequently effacement of podocyte foot processes was noted in the kidneys of D2GN but not B6GN mice. These defects are likely to be involved in the integrin signaling pathway.
  
  
  CONCLUSIONS
  This study suggests that Tenc1 contributes to the maintenance of GBM structures and that the genetic background influences the severity of nephrotic syndrome.
authors.affiliation: Laboratory of Animal Models for Human Diseases, National Institute of Biomedical Innovation, Ibaraki, Japan.
authors.forename: Kozue
authors.initials: K
authors.lastname: Uchio-Yamada
authors.forename: Kyoko
authors.initials: K
authors.lastname: Sawada
authors.forename: Kotaro
authors.initials: K
authors.lastname: Tamura
authors.forename: Sumie
authors.initials: S
authors.lastname: Katayama
authors.forename: Youko
authors.initials: Y
authors.lastname: Monobe
authors.forename: Yoshie
authors.initials: Y
authors.lastname: Yamamoto
authors.forename: Atsuo
authors.initials: A
authors.lastname: Ogura
authors.forename: Noboru
authors.initials: N
authors.lastname: Manabe
mesh_terms: D048868:Adaptor Proteins, Signal Transducing / Q000378:metabolism; D000818:Animals; D015153:Blotting, Western; D024141:Collagen Type IV / Q000378:metabolism; D003598:Cytoskeletal Proteins / Q000378:metabolism; D050533:Glomerular Basement Membrane / Q000378:metabolism* / Q000473:pathology / Q000648:ultrastructure; D005921:Glomerulonephritis / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D039201:Integrin alpha3beta1 / Q000378:metabolism; D007678:Kidney Glomerulus / Q000378:metabolism* / Q000473:pathology / Q000648:ultrastructure; D007797:Laminin / Q000378:metabolism; D008565:Membrane Proteins / Q000378:metabolism; D051379:Mice; D008810:Mice, Inbred C57BL; D008811:Mice, Inbred DBA; D008813:Mice, Inbred ICR; D018345:Mice, Knockout; D046529:Microscopy, Electron, Transmission; D008856:Microscopy, Fluorescence; D009404:Nephrotic Syndrome / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D010749:Phosphoprotein Phosphatases / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D050199:Podocytes / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D011507:Proteinuria / Q000652:urine; D013045:Species Specificity; D000070876:Tensins
chemical_list: D048868:Adaptor Proteins, Signal Transducing; C114545:CD2-associated protein; D024141:Collagen Type IV; D003598:Cytoskeletal Proteins; D039201:Integrin alpha3beta1; D007797:Laminin; D008565:Membrane Proteins; D000070876:Tensins; C113267:nephrin; D010749:Phosphoprotein Phosphatases; C511707:Tns2 protein, mouse