Tensin2-deficient mice on FVB/N background develop severe glomerular disease

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Author(s)

Abstract

Tensin2 (Tns2) is an essential component for the maintenance of glomerular basement membrane (GBM) structures. <i>Tns2-</i>deficient mice were previously shown to develop mild glomerular injury on a DBA/2 background, but not on a C57BL/6J or a 129/SvJ background, suggesting that glomerular injury by the deletion of <i>Tns2</i> was strongly dependent on the genetic background. To further understand the mechanisms for the onset and the progression of glomerular injury by the deletion of <i>Tns2</i>, we generated <i>Tns2-</i>deficient mice on an FVB/N (FVB) strain, which is highly sensitive to glomerular disease. <i>Tns2-</i>deficient mice on FVB (FVBGN) developed severe nephrotic syndrome, and female FVBGN mice died within 8 weeks. Ultrastructural analysis revealed that FVBGN mice exhibited severe glomerular defects with mesangial process invasion of glomerular capillary tufts, lamination and thickening of the GBM and subsequent podocyte foot process effacement soon after birth. Aberrant laminin components containing α1, α2 and β1 chains, which are normally expressed in the mesangium, accumulated in the GBM of FVBGN, suggesting that these components originated from mesangial cells that invaded glomerular capillary tufts. Compared to <i>Tns2-</i>deficient mice on the other backgrounds in previous reports, FVBGN mice developed earlier onset of glomerular defects and rapid progression of renal failure. Thus, this study further extended our understanding of the possible genetic background effect on the deterioration of nephrotic syndrome by <i>Tns2</i> deficiency.

Journal

  • Journal of Veterinary Medical Science

    Journal of Veterinary Medical Science 78(5), 811-818, 2016

    JAPANESE SOCIETY OF VETERINARY SCIENCE

Codes

  • NII Article ID (NAID)
    130005154309
  • NII NACSIS-CAT ID (NCID)
    AA10796138
  • Text Lang
    ENG
  • ISSN
    0916-7250
  • NDL Article ID
    027450199
  • NDL Call No.
    Z18-350
  • Data Source
    NDL  J-STAGE 
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