Neural retina leucine zipper (NRL) is essential for the development of rods. The Nrl knockout (Nrl−/−) mouse develops a cone-rich retina which facilitates investigations of cone biology. Previously, we identified the endoplasmic reticulum (ER) stress response gene ATF6 as a cause of cone dysfunction in people. Here, we investigated Atf6’s influence on mouse cones in the cone-dominant Nrl−/− mouse. We find that cone development appears unaffected, but the cone-dominant Nrl−/− retina is highly vulnerable to ER stress-induced damage.

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Vulnerability of the Nrl−/− Cone-Dominant Retina to Endoplasmic Reticulum Stress

  • Angela Galdamez,
  • Masako Le,
  • Wei-Chieh Jerry Chiang,
  • Eun-Jin Lee,
  • Jonathan H. Lin

摘要

Neural retina leucine zipper (NRL) is essential for the development of rods. The Nrl knockout (Nrl−/−) mouse develops a cone-rich retina which facilitates investigations of cone biology. Previously, we identified the endoplasmic reticulum (ER) stress response gene ATF6 as a cause of cone dysfunction in people. Here, we investigated Atf6’s influence on mouse cones in the cone-dominant Nrl−/− mouse. We find that cone development appears unaffected, but the cone-dominant Nrl−/− retina is highly vulnerable to ER stress-induced damage.