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ATF6 is essential for human cone photoreceptor development.

Heike Kroeger1, Julia M D Grandjean2, Wei-Chieh Jerry Chiang3

  • 1Department of Cellular Biology, Franklin College of Arts and Sciences, University of Georgia, Athens, GA 30601; heike.kroeger@uga.edu jlinn@stanford.edu.

Proceedings of the National Academy of Sciences of the United States of America
|September 25, 2021
PubMed
Summary

Activating Transcription Factor 6 (ATF6) is crucial for human cone photoreceptor development. Targeting ATF6 signaling may offer a therapeutic strategy for achromatopsia and other blinding retinal diseases.

Keywords:
ATF6 signalingachromatopsiacone photoreceptorsretinal organoidsstem cell biology

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Area of Science:

  • Ophthalmology
  • Genetics
  • Cell Biology

Background:

  • Endoplasmic reticulum (ER) stress and Unfolded Protein Response (UPR) signaling are implicated in human diseases.
  • Loss-of-function variants in Activating Transcription Factor 6 (ATF6), a UPR regulator, cause congenital vision loss, including achromatopsia, through unknown mechanisms.

Purpose of the Study:

  • To investigate the role of ATF6 in human cone photoreceptor development and the pathomechanisms of ATF6-associated achromatopsia.
  • To explore the therapeutic potential of targeting ATF6 signaling for blinding retinal diseases.

Main Methods:

  • Generated retinal organoids from induced pluripotent stem cells of achromatopsia patients with ATF6 variants and from gene-edited ATF6-null human embryonic stem cells.
  • Utilized adaptive optics retinal imaging in achromatopsia patients carrying ATF6 variants.
  • Tested a small molecule ATF6 signaling agonist on patient-derived retinal organoids.

Main Results:

  • Retinal organoids lacking functional ATF6 failed to form cone structures and showed reduced cone phototransduction gene expression, while rod development was normal.
  • Patient imaging revealed an absence of cone structures, mirroring organoid findings.
  • An ATF6 agonist partially restored transcriptional activity in some disease variants and promoted cone growth and gene expression in patient organoids.

Conclusions:

  • ATF6 is essential for human cone development.
  • Pharmacologic targeting of the ATF6 pathway shows potential for promoting cone development and treating blinding retinal diseases like achromatopsia.