Loss of alpha3/alpha4(IV) collagen from the glomerular basement membrane induces a strain-dependent isoform switch to alpha5alpha6(IV) collagen associated with longer renal survival in Col4a3-/- Alport mice.

@article{Kang2006LossOA,
  title={Loss of alpha3/alpha4(IV) collagen from the glomerular basement membrane induces a strain-dependent isoform switch to alpha5alpha6(IV) collagen associated with longer renal survival in Col4a3-/- Alport mice.},
  author={Jeong Suk Kang and Xu-ping Wang and Jeffrey H Miner and Roy Morello and Yoshikazu Sado and Dale R. Abrahamson and Dorin B Borza},
  journal={Journal of the American Society of Nephrology : JASN},
  year={2006},
  volume={17 7},
  pages={
          1962-9
        }
}
Mutations in COL4A3/4/5 genes that affect the normal assembly of the alpha3/4/5(IV) collagen network in the glomerular basement membrane (GBM) cause Alport syndrome. Patients progress to renal failure at variable rates that are determined by the underlying mutation and putative modifier genes. Col4a3(-/-) mice, a model for autosomal recessive Alport syndrome, progress to renal failure significantly slower on the C57BL/6 than on the 129X1/Sv background. Reported here is a novel strain-specific… CONTINUE READING

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