{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T20:03:50Z","timestamp":1788897830138,"version":"build-2803163510"},"reference-count":101,"publisher":"American Society for Clinical Investigation","issue":"12","license":[{"start":{"date-parts":[[2025,6,16]],"date-time":"2025-06-16T00:00:00Z","timestamp":1750032000000},"content-version":"vor","delay-in-days":38,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Molloy Family Research and Innovation Fund at BCH","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}]},{"name":"NIH","award":["R35GM142553"],"award-info":[{"award-number":["R35GM142553"]}]},{"name":"The Jane Coffin Childs Fund for Medical","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}]},{"name":"F.M. 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ADOA is primarily caused by mutations in the optic atrophy type 1 (\n                    <jats:italic>OPA1<\/jats:italic>\n                    ) gene, which encodes a conserved GTPase important for mitochondrial inner membrane dynamics. To date, the disease mechanism remains unclear, and no therapies are available. We generated a mouse model carrying the pathogenic\n                    <jats:italic>\n                      Opa1\n                      <jats:sup>R290Q\/+<\/jats:sup>\n                    <\/jats:italic>\n                    allele that recapitulated key features of human ADOA, including mitochondrial defects, age-related RGC loss, optic nerve degeneration, and reduced RGC functions. We identified sterile alpha and TIR motif containing 1 (SARM1), a neurodegeneration switch, as a key driver of RGC degeneration in these mice.\n                    <jats:italic>Sarm1<\/jats:italic>\n                    KO nearly completely suppressed all the degeneration phenotypes without reversing mitochondrial fragmentation. Additionally, we show that a portion of SARM1 localized within the mitochondrial intermembrane space. These findings indicated that SARM1 was activated downstream of mitochondrial dysfunction in ADOA, highlighting it as a promising therapeutic target.\n                  <\/jats:p>","DOI":"10.1172\/jci191315","type":"journal-article","created":{"date-parts":[[2025,5,9]],"date-time":"2025-05-09T13:51:04Z","timestamp":1746798664000},"source":"Crossref","is-referenced-by-count":10,"title":["SARM1 loss protects retinal ganglion cells in a mouse model of autosomal dominant optic atrophy"],"prefix":"10.1172","volume":"135","author":[{"given":"Chen","family":"Ding","sequence":"first","affiliation":[{"name":"F.M. Kirby Neurobiology Center, Boston Children\u2019s Hospital, Boston, Massachusetts, USA."},{"name":"Department of Neurobiology, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Papa S.","family":"Ndiaye","sequence":"additional","affiliation":[{"name":"F.M. 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Kirby Neurobiology Center, Boston Children\u2019s Hospital, Boston, Massachusetts, USA."},{"name":"Department of Neurology, Harvard Medical School, Boston, Massachusetts, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle Y.","family":"Fry","sequence":"additional","affiliation":[{"name":"Department of Molecular Biology, Massachusetts General Hospital, Boston, Massachusetts, USA."},{"name":"Department of Genetics, Blavatnik Institute, Harvard Medical School, Boston, Massachusetts, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-5500-5286","authenticated-orcid":true,"given":"Jincheng","family":"Gong","sequence":"additional","affiliation":[{"name":"F.M. Kirby Neurobiology Center, Boston Children\u2019s Hospital, Boston, Massachusetts, USA."},{"name":"Department of Neurobiology, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophia R.","family":"Wienbar","sequence":"additional","affiliation":[{"name":"F.M. Kirby Neurobiology Center, Boston Children\u2019s Hospital, Boston, Massachusetts, USA."},{"name":"Department of Neurology, Harvard Medical School, Boston, Massachusetts, USA."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Whitney","family":"Gibbs","sequence":"additional","affiliation":[{"name":"F.M. Kirby Neurobiology Center, Boston Children\u2019s Hospital, Boston, Massachusetts, USA."},{"name":"Department of Neurobiology, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Morquette","sequence":"additional","affiliation":[{"name":"F.M. 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