Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Research Highlight
  • Published:

Ciliopathies

Inpp5e links lipids, cysts and cilia

Nature Reviews Nephrology volume 12, page 508 (2016)Cite this article

Subjects

Mutations in INPP5E cause Joubert syndrome, a ciliopathy that affects various organs and causes renal cysts; however, the mechanisms by which INPP5E dysfunction leads to defective ciliogenesis and cystic kidneys are unclear. In a new study, Ying Cao and colleagues show that Inpp5e modulates ciliogenesis and cystogenesis by regulating basal body docking and cell polarity by controlling the spatial distribution of phosphoinositides (PIs), which are phosphorylated derivatives of phosphatidylinositol (PtdIns). “Previous findings indicated that Inpp5e was required for cilia maintenance but not assembly,” explains Cao. “Our work shows that Inpp5e functions in both ciliogenesis and cystogenesis.”

In zebrafish, loss of inpp5e led to renal cysts, ciliogenesis defects, impaired apical docking of basal bodies and redistribution of apical F-actin towards the lateral membranes of the pronephric epithelium. In addition, the apical localization of PtdIns(4,5)P2 was lost, whereas PtdIns(3,4,5)P3 was ectopically localized to the apical membrane.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on SpringerLink
  • Instant access to the full article PDF.

USD 39.95

Prices may be subject to local taxes which are calculated during checkout

References

  1. Xu, W. et al. The Joubert syndrome protein Inpp5e controls ciliogenesis by regulating phosphoinositides at the apical membrane. J. Am. Soc. Nephrol. http://dx.doi.org/10.1681/ASN.2015080906 (2016)

Download references

Authors
  1. Andrea Aguilar
    View author publications

    Search author on:PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Aguilar, A. Inpp5e links lipids, cysts and cilia. Nat Rev Nephrol 12, 508 (2016). https://doi.org/10.1038/nrneph.2016.120

Download citation

  • Published:

  • Issue date:

  • DOI: https://doi.org/10.1038/nrneph.2016.120

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

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