{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T09:32:38Z","timestamp":1740130358535,"version":"3.37.3"},"reference-count":30,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2023,5,5]],"date-time":"2023-05-05T00:00:00Z","timestamp":1683244800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Korean Health Technology"},{"name":"Ministry of Health & Welfare","award":["HI14C3484","HI20C0039"],"award-info":[{"award-number":["HI14C3484","HI20C0039"]}]},{"DOI":"10.13039\/501100014188","name":"Ministry of Science and ICT","doi-asserted-by":"publisher","award":["NRF-2016R1A5A2007009","NRF-2020M3H4A1A03084600","NRF-2021R1A2C1007793"],"award-info":[{"award-number":["NRF-2016R1A5A2007009","NRF-2020M3H4A1A03084600","NRF-2021R1A2C1007793"]}],"id":[{"id":"10.13039\/501100014188","id-type":"DOI","asserted-by":"publisher"}]},{"name":"National Research Facilities"},{"DOI":"10.13039\/100009122","name":"Ministry of Education","doi-asserted-by":"publisher","award":["NRF-2021R1A6C101A425","NRF-2022R1A6C103A720"],"award-info":[{"award-number":["NRF-2021R1A6C101A425","NRF-2022R1A6C103A720"]}],"id":[{"id":"10.13039\/100009122","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,9,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>The duplication of the peripheral myelin protein 22 (PMP22) gene causes a demyelinating type of neuropathy, commonly known as Charcot\u2013Marie\u2013Tooth disease type 1A (CMT1A). Development of effective drugs for CMT1A still remains as an unmet medical need.<\/jats:p>\n               <jats:p>In the present study, we assessed the role of the transforming growth factor beta 4 (TGF\u03b24)\/Nodal axis in the pathogenesis of CMT1A.<\/jats:p>\n               <jats:p>First, we identified PMP22 overexpression-induced Nodal expression in Schwann cells, which might be one of the downstream effectors in CMT1A. Administration of Nodal protein at the developmental stage of peripheral nerves induced the demyelinating phenotype in vivo. Second, we further isolated TGF\u03b24 as an antagonist that could abolish Nodal-induced demyelination. Finally, we developed a recombinant TGF\u03b24\u2013fragment crystallizable (Fc) fusion protein, CX201, and demonstrated that its application had promyelinating efficacy in Schwann cells. CX201 administration improved the demyelinating phenotypes of CMT1A mouse models at both pre-symptomatic and post-symptomatic stages.<\/jats:p>\n               <jats:p>These results suggest that the TGF\u03b24\/Nodal axis plays a crucial role in the pathogenesis of CMT1A and might be a potential therapeutic target for CMT1A.<\/jats:p>","DOI":"10.1093\/brain\/awad147","type":"journal-article","created":{"date-parts":[[2023,5,5]],"date-time":"2023-05-05T05:15:22Z","timestamp":1683263722000},"page":"3608-3615","source":"Crossref","is-referenced-by-count":0,"title":["TGF\u03b24 alleviates the phenotype of Charcot\u2013Marie\u2013Tooth disease type 1A"],"prefix":"10.1093","volume":"146","author":[{"given":"Hyeonjin","family":"Jeon","sequence":"first","affiliation":[{"name":"Department of Translational Biomedical Sciences, Graduate School of Dong-A University , Busan 49201 , Korea"},{"name":"Dementia Research Group, Korea Brain Research Institute , Daegu 41062 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"So Young","family":"Jang","sequence":"additional","affiliation":[{"name":"Department of Translational Biomedical Sciences, Graduate School of Dong-A University , Busan 49201 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Geon","family":"Kwak","sequence":"additional","affiliation":[{"name":"Department of Health Sciences and Technology, SAIHST, Sungkyunkwan University , Seoul 06351 , Korea"},{"name":"BioMedicine Lab., CKD Research Institute, ChongKunDang Pharm. , Yongin 16995 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yong Weon","family":"Yi","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, College of Medicine, Dankook University , Cheonan 31116 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mi-Hyeon","family":"You","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine , Seoul 05505 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Na Young","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Translational Biomedical Sciences, Graduate School of Dong-A University , Busan 49201 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ju Hee","family":"Jo","sequence":"additional","affiliation":[{"name":"Department of Translational Biomedical Sciences, Graduate School of Dong-A University , Busan 49201 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ji Won","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Translational Biomedical Sciences, Graduate School of Dong-A University , Busan 49201 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hye Ji","family":"Jang","sequence":"additional","affiliation":[{"name":"Department of Translational Biomedical Sciences, Graduate School of Dong-A University , Busan 49201 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sun-Young","family":"Jeong","sequence":"additional","affiliation":[{"name":"BioMedicine Lab., CKD Research Institute, ChongKunDang Pharm. , Yongin 16995 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seung Kee","family":"Moon","sequence":"additional","affiliation":[{"name":"BioMedicine Lab., CKD Research Institute, ChongKunDang Pharm. , Yongin 16995 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hyun Myung","family":"Doo","sequence":"additional","affiliation":[{"name":"Department of Health Sciences and Technology, SAIHST, Sungkyunkwan University , Seoul 06351 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Minyeop","family":"Nahm","sequence":"additional","affiliation":[{"name":"Dementia Research Group, Korea Brain Research Institute , Daegu 41062 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Donghoon","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Translational Biomedical Sciences, Graduate School of Dong-A University , Busan 49201 , Korea"},{"name":"Department of Pharmacology, College of Medicine, Dong-A University , Busan 49201 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jong Wook","family":"Chang","sequence":"additional","affiliation":[{"name":"Stem Cell and Regenerative Medicine Institute, Samsung Medical Center , Seoul 06351 , Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Byung-Ok","family":"Choi","sequence":"additional","affiliation":[{"name":"Department of Health Sciences and Technology, SAIHST, Sungkyunkwan University , Seoul 06351 , Korea"},{"name":"Stem Cell and Regenerative Medicine Institute, Samsung Medical Center , Seoul 06351 , Korea"},{"name":"Department of Neurology, Samsung Medical Center, Sungkyunkwan 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