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U.S.A."],"published-print":{"date-parts":[[2021,9,28]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>\n                    Inactivating genetic variants in the Unfolded Protein Response (UPR) regulator\n                    <jats:italic>Activating Transcription Factor 6 (ATF6)<\/jats:italic>\n                    result in loss of color vision, which is linked to dysfunctional cone photoreceptors and the development of achromatopsia. The pathomechanims involving the lack of functional ATF6 remain unknown; retinal organoids from patient induced pluripotent stem cells and\n                    <jats:italic>ATF6<\/jats:italic>\n                    null hESCs were generated to elucidate this question. Data from this study identified an unexpected UPR-independent role for ATF6 signaling during cone photoreceptor differentiation in human retinal development, identifying a pathogenic mechanism underlying ATF6-associated achromatopsia. It is believed that pharmacologic targeting of ATF6 may present a viable treatment option for color blinding diseases at early stages of retinal development.\n                  <\/jats:p>","DOI":"10.1073\/pnas.2103196118","type":"journal-article","created":{"date-parts":[[2021,9,24]],"date-time":"2021-09-24T15:56:00Z","timestamp":1632498960000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":53,"title":["ATF6 is essential for human cone photoreceptor development"],"prefix":"10.1073","volume":"118","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7726-7748","authenticated-orcid":false,"given":"Heike","family":"Kroeger","sequence":"first","affiliation":[{"name":"Department of Cellular Biology, Franklin College of Arts and Sciences, University of Georgia, Athens, GA 30601;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia M. 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