News & Views | Published:


Autosomal dominant PKD gets an atomic map

Nature Reviews Nephrologyvolume 14pages725726 (2018) | Download Citation

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.

Access optionsAccess options

Rent or Buy article

Get time limited or full article access on ReadCube.


All prices are NET prices.


  1. 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).

  2. 2.

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

  3. 3.

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

  4. 4.

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

  5. 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).

  6. 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).

  7. 7.

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

  8. 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).

  9. 9.

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

Download references


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.

Author information


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

    • Paul A. Welling


  1. Search for Paul A. Welling in:

Competing interests

The author declares no competing interests.

Corresponding author

Correspondence to Paul A. Welling.

About this article

Publication history



Newsletter Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing