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POLYCYSTIC KIDNEY DISEASE

Autosomal dominant PKD gets an atomic map

Nature Reviews Nephrology volume 14, pages 725–726 (2018)Cite this article

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The polycystin complex structure has been solved at near-atomic resolution. Its surprising architecture provides new insights into the transient receptor potential (TRP) family of cation channels and the pathogenesis of autosomal dominant polycystic kidney disease. This discovery should have a transformative impact on the development of treatment strategies to cure the disease.

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Fig. 1: A top-down view of polycystin complexes.

References

  1. Harris, P. C. & Torres, V. E. Genetic mechanisms and signaling pathways in autosomal dominant polycystic kidney disease. J. Clin. Invest. 124, 2315–2324 (2014).

    Article  CAS  Google Scholar 

  2. Su, Q. et al. Structure of the human PKD1/PKD2 complex. Science 361, eaat9819 (2018).

    Article  Google Scholar 

  3. Thonghin, N. et al. Cryo-electron microscopy of membrane proteins. Methods 147, 176–186 (2018).

    Article  CAS  Google Scholar 

  4. Winn, M. P. et al. A mutation in the TRPC6 cation channel causes familial focal segmental glomerulosclerosis. Science 308, 1801–1804 (2005).

    Article  CAS  Google Scholar 

  5. Voets, T. et al. TRPM6 forms the Mg2+ influx channel involved in intestinal and renal Mg2+ absorption. J. Biol. Chem. 279, 19–25 (2004).

    Article  CAS  Google Scholar 

  6. Schlingmann, K. P. et al. Hypomagnesemia with secondary hypocalcemia is caused by mutations in TRPM6, a new member of the TRPM gene family. Nat. Genet. 31, 166–170 (2002).

    Article  CAS  Google Scholar 

  7. Hulse, R. E. et al. Cryo-EM structure of the polycystin 2-l1 ion channel. eLife 7, e36931 (2018).

    Article  Google Scholar 

  8. Liu, X. et al. Polycystin-2 is an essential ion channel subunit in the primary cilium of the renal collecting duct epithelium. eLife 7, e33183 (2018).

    Article  Google Scholar 

  9. Grieben, M. et al. Structure of the polycystic kidney disease TRP channel polycystin-2 (PC2). Nat. Struct. Mol. Biol. 24, 114–122 (2017).

    Article  CAS  Google Scholar 

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Acknowledgements

The author is supported by grants from the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), including to the Baltimore Polycystic Kidney Disease (PKD) Research and Clinical Core Center.

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  1. University of Maryland Medical School, Baltimore, MD, USA

    Paul A. Welling

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  1. Paul A. Welling
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Correspondence to Paul A. Welling.

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The author declares no competing interests.

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Welling, P.A. Autosomal dominant PKD gets an atomic map. Nat Rev Nephrol 14, 725–726 (2018). https://doi.org/10.1038/s41581-018-0066-7

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