{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T16:16:18Z","timestamp":1777392978222,"version":"3.51.4"},"reference-count":50,"publisher":"SAGE Publications","issue":"4","license":[{"start":{"date-parts":[[2024,7,15]],"date-time":"2024-07-15T00:00:00Z","timestamp":1721001600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Alzheimer\u2019s Disease"],"published-print":{"date-parts":[[2024,7,18]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>\n                      Cerebral autosomal-dominant arteriopathy with subcortical infarction and leukoencephalopathy (CADASIL) is an inherited small-vessel disease that affects the white matter of the brain. Recent studies have confirmed that the deposition of NOTCH3\n                      <jats:sup>ECD<\/jats:sup>\n                      is the main pathological basis of CADASIL; however, whether different mutations present the same pathological characteristics remains to be further studied. Some studies have found that mitochondrial dysfunction is related to CADASIL; however, the specific effects of NOTCH3\n                      <jats:sup>ECD<\/jats:sup>\n                      on mitochondrial remain to be determined.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective:<\/jats:title>\n                    <jats:p>We aimed to explore the role of mitochondrial dysfunction in CADASIL.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>We established transgenic human embryonic kidney-293T cell models (involving alterations in cysteine and non-cysteine residues) via lentiviral transfection. Mitochondrial function and structure were assessed using flow cytometry and transmission electron microscopy, respectively. Mitophagy was assessed using western blotting and immunofluorescence.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      We demonstrated that NOTCH3\n                      <jats:sup>ECD<\/jats:sup>\n                      deposition affects mitochondrial morphology and function, and that its protein levels are significantly correlated with mitochondrial quality and can directly bind to mitochondria. Moreover, NOTCH3\n                      <jats:sup>ECD<\/jats:sup>\n                      deposition promoted the induction of autophagy and mitophagy. However, these processes were impaired, leading to abnormal mitochondrial accumulation.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>\n                      This study revealed a common pathological feature of NOTCH3\n                      <jats:sup>ECD<\/jats:sup>\n                      deposition caused by different NOTCH3 mutations and provided new insights into the role of NOTCH3\n                      <jats:sup>ECD<\/jats:sup>\n                      in mitochondrial dysfunction and mitophagy.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.3233\/jad-240273","type":"journal-article","created":{"date-parts":[[2024,7,19]],"date-time":"2024-07-19T11:49:48Z","timestamp":1721389788000},"page":"1299-1314","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":2,"title":["Mutant NOTCH3\n                    <sup>ECD<\/sup>\n                    Triggers Defects in Mitochondrial Function and Mitophagy in CADASIL Cell Models"],"prefix":"10.1177","volume":"100","author":[{"given":"Wan","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Neurology, Zhengzhou University People\u2019s Hospital, Henan Provincial People\u2019s Hospital, Zhengzhou, Henan, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhenping","family":"Gong","sequence":"additional","affiliation":[{"name":"Department of Neurology, Xinxiang medical university, Henan Provincial People\u2019s Hospital, Zhengzhou, Henan, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yadan","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Neurology, Henan University People\u2019s Hospital, Henan Provincial People\u2019s Hospital, Zhengzhou, Henan, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ying","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Neurology, Zhengzhou University People\u2019s Hospital, Henan Provincial People\u2019s Hospital, Zhengzhou, Henan, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yaru","family":"Lu","sequence":"additional","affiliation":[{"name":"Department of Neurology, Zhengzhou University People\u2019s Hospital, Henan Provincial People\u2019s Hospital, Zhengzhou, Henan, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ruihua","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Neurology, Zhengzhou University People\u2019s Hospital, Henan Provincial People\u2019s Hospital, Zhengzhou, Henan, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haohan","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Neurology, Zhengzhou University People\u2019s Hospital, Henan Provincial People\u2019s Hospital, Zhengzhou, Henan, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Junkui","family":"Shang","sequence":"additional","affiliation":[{"name":"Department of Neurology, Zhengzhou University People\u2019s Hospital, Henan Provincial People\u2019s Hospital, Zhengzhou, Henan, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jiewen","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Neurology, Zhengzhou University People\u2019s Hospital, Henan Provincial People\u2019s Hospital, Zhengzhou, Henan, 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