{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T18:15:21Z","timestamp":1781892921479,"version":"3.54.5"},"reference-count":57,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2022,6,23]],"date-time":"2022-06-23T00:00:00Z","timestamp":1655942400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["31971140"],"award-info":[{"award-number":["31971140"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["21907033"],"award-info":[{"award-number":["21907033"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["faseb.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The FASEB Journal"],"published-print":{"date-parts":[[2022,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Harmonin is a protein containing multiple PDZ domains and is required for the development and maintenance of hair cell stereocilia and brush border microvilli. Mutations in the\n                    <jats:italic>USH1C<\/jats:italic>\n                    gene can cause Usher syndrome type 1C, a severe inheritable disease characterized by the loss of hearing and vision. Here, by solving the high\u2010resolution crystal structure of Harmonin PDZ2 and coiled\u2010coil domains in a complex with the tail of cadherin\u2010related family member 2, we demonstrated that mutations located in the Harmonin PDZ2 domain and found in patients could affect its stability, and thus, the target binding capability. The structure also implies that the coiled\u2010coil domain could form antiparallel dimers under high concentrations, possibly when Harmonin underwent liquid\u2013liquid phase separation in the upper tip\u2010link density in hair cell stereocilia or microvilli of enterocytes of the intestinal epithelium. The crystal structure, together with the biochemical analysis, provided mechanistic implications for Harmonin mutations causing Usher syndrome, non\u2010syndromic deafness, or enteropathy.\n                  <\/jats:p>","DOI":"10.1096\/fj.202200403rr","type":"journal-article","created":{"date-parts":[[2022,6,24]],"date-time":"2022-06-24T02:19:35Z","timestamp":1656037175000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Structure of the Harmonin\n                    <scp>PDZ2<\/scp>\n                    and coiled\u2010coil domains in a complex with\n                    <scp>CDHR2<\/scp>\n                    tail and its implications"],"prefix":"10.1096","volume":"36","author":[{"given":"Wenxia","family":"Yan","sequence":"first","affiliation":[{"name":"School of Biology and Biological Engineering South China University of Technology  Guangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guanhao","family":"Chen","sequence":"additional","affiliation":[{"name":"Division of Cell, Developmental and Integrative Biology, School of Medicine South China University of Technology  Guangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8921-1626","authenticated-orcid":false,"given":"Jianchao","family":"Li","sequence":"additional","affiliation":[{"name":"Division of Cell, Developmental and Integrative Biology, School of Medicine South China University of Technology  Guangzhou China"},{"name":"Department of Otorhinolaryngology, Guangzhou First People's Hospital, School of Medicine South China University of Technology  Guangzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,6,23]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00018-009-0254-5"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cub.2021.04.005"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.cb.08.110192.001105"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.cb.01.110185.001233"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-cellbio-100814-125234"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.201407015"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.3109\/01913123.2010.537438"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1155\/2001\/743925"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.cb.08.110192.001353"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2014.11.020"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-neuro-070918-050428"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.heares.2020.107932"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2014.01.067"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pbio.3001463"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature06091"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature11590"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0911385107"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.devcel.2015.12.020"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1702251114"},{"issue":"15","key":"e_1_2_10_21_1","first-page":"864","article-title":"Myosin 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