Molecular and functional defects in kidneys of mice lacking collagen alpha 3(IV): implications for Alport syndrome

@article{Miner1996MolecularAF,
  title={Molecular and functional defects in kidneys of mice lacking collagen alpha 3(IV): implications for Alport syndrome},
  author={Jeffrey H Miner and Joshua R Sanes},
  journal={The Journal of Cell Biology},
  year={1996},
  volume={135},
  pages={1403 - 1413}
}
Collagen IV is a major structural component of all basal laminae (BLs). Six collagen IV alpha chains are present in mammals; alpha 1 and alpha 2(IV) are broadly expressed in embryos and adults, whereas alpha 3-6(IV) are restricted to a defined subset of BLs. In the glomerular BL of the kidney, the alpha 1 and alpha 2(IV) chains are replaced by the alpha 3-5(IV) chains as development proceeds. In humans, mutation of the collagen alpha 3, alpha 4, or alpha 5(IV) chain genes results in a delayed… CONTINUE READING

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