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The EH Binding protein EHBP1 operates in a ciliary functional module affected by INPP5E dysfunction.

Kae R Whiting1, Mariam Aslanyan1, Imke Peters1

  • 1Department of Human Genetics, Research Institute for Medical Innovation, Radboud University Medical Center, Nijmegen, Netherlands.

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|March 10, 2026
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Summary

Inositol polyphosphate-5-phosphatase E (INPP5E) regulates primary cilia. New research identifies EH binding protein 1 (EHBP1) as a ciliary protein interacting with INPP5E, offering insights into retinal ciliopathies.

Keywords:
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Area of Science:

  • Cell Biology
  • Molecular Biology
  • Genetics

Background:

  • Primary cilia are crucial for cellular function.
  • Inositol polyphosphate-5-phosphatase E (INPP5E) is a key ciliary protein.
  • Dysfunction of INPP5E is linked to retinal ciliopathies.

Purpose of the Study:

  • To identify proteins interacting with INPP5E using proximity labeling.
  • To investigate the role of EH binding protein 1 (EHBP1) in primary cilia and photoreceptors.
  • To understand the impact of INPP5E dysfunction on EHBP1 localization and retinal ciliopathies.

Main Methods:

  • Proximity labeled proteomics of INPP5E.
  • Immunofluorescence microscopy in human fibroblasts, RPE cells, and retinal organoids.
  • CRISPR/Cas9 gene editing to create INPP5E knock-out models.

Main Results:

  • EHBP1 was identified as a ciliary protein localizing to the basal body and ciliary compartment.
  • EHBP1 localizes to the outer membrane of developing photoreceptors.
  • INPP5E dysfunction alters EHBP1 localization in both patient-derived fibroblasts and knock-out retinal organoids.

Conclusions:

  • EHBP1 functions at the primary cilium and photoreceptors and is regulated by INPP5E.
  • This interaction provides insights into the pathogenesis of retinal ciliopathies.
  • EHBP1 is a potential candidate protein for retinitis pigmentosa and other retinal ciliopathies.