Publication: Transgenic zebrafish modeling low-molecular-weight proteinuria and lysosomal storage diseases
Transgenic zebrafish modeling low-molecular-weight proteinuria and lysosomal storage diseases
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Chen, Z., Luciani, A., Mateos, J. M., Barmettler, G., Giles, R. H., Neuhauss, S. C. F., & Devuyst, O. (2020). Transgenic zebrafish modeling low-molecular-weight proteinuria and lysosomal storage diseases. Kidney International, 97(6), 1150–1163. https://doi.org/10.1016/j.kint.2019.11.016
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Epithelial cells lining the proximal tubule of the kidneyreabsorb and metabolize most of thefiltered low-molecular-weight proteins through receptor-mediatedendocytosis and lysosomal processing. Congenital andacquired dysfunctions of the proximal tubule areconsistently reflected by the inappropriate loss of solutesincluding low-molecular-weight proteins in the urine. Thezebrafish pronephros shares individual functionalsegments with the human nephron, including lrp2a/megalin-dependent endocytic transport processes of theproximal tubule.
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Chen, Z., Luciani, A., Mateos, J. M., Barmettler, G., Giles, R. H., Neuhauss, S. C. F., & Devuyst, O. (2020). Transgenic zebrafish modeling low-molecular-weight proteinuria and lysosomal storage diseases. Kidney International, 97(6), 1150–1163. https://doi.org/10.1016/j.kint.2019.11.016