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Tensin2-deficient mice on FVB/N background develop severe glomerular disease
- Source :
- The Journal of Veterinary Medical Science
- Publication Year :
- 2016
- Publisher :
- Japanese Society of Veterinary Science, 2016.
-
Abstract
- Tensin2 (Tns2) is an essential component for the maintenance of glomerular basement membrane (GBM) structures. Tns2-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 Tns2 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 Tns2, we generated Tns2-deficient mice on an FVB/N (FVB) strain, which is highly sensitive to glomerular disease. Tns2-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 Tns2-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 Tns2 deficiency.
- Subjects :
- Male
0301 basic medicine
medicine.medical_specialty
Nephrotic Syndrome
Kidney Glomerulus
Mice, Inbred Strains
urologic and male genital diseases
Mice
03 medical and health sciences
Species Specificity
Laminin
Tensins
Internal medicine
glomerular basement membrane (GBM)
Tenc1)
Glomerular Basement Membrane
Deficient mouse
medicine
Animals
Glomerular disease
Full Paper
General Veterinary
biology
Podocytes
urogenital system
business.industry
Glomerular basement membrane
Glomerulonephritis
medicine.disease
female genital diseases and pregnancy complications
Mice, Inbred C57BL
030104 developmental biology
medicine.anatomical_structure
Endocrinology
Mice, Inbred DBA
Mesangium
biology.protein
FVB/N
tensin2 (Tns2
Female
Anatomy
business
Nephrotic syndrome
ICR-derived glomerulonephritis (ICGN) mouse
Subjects
Details
- ISSN :
- 13477439 and 09167250
- Volume :
- 78
- Database :
- OpenAIRE
- Journal :
- Journal of Veterinary Medical Science
- Accession number :
- edsair.doi.dedup.....0e425c08fb902c56bb36d59db57faa66