{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T01:44:09Z","timestamp":1781833449601,"version":"3.54.5"},"reference-count":33,"publisher":"Rockefeller University Press","issue":"5","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2012,5,28]]},"abstract":"<jats:p>Dysfunction of the basement membrane protein QBRICK provokes Fraser syndrome, which results in renal dysmorphogenesis, cryptophthalmos, syndactyly, and dystrophic epidermolysis bullosa through unknown mechanisms. Here, we show that integrin \u03b18\u03b21 binding to basement membranes was significantly impaired in Qbrick-null mice. This impaired integrin \u03b18\u03b21 binding was not a direct consequence of the loss of QBRICK, which itself is a ligand of integrin \u03b18\u03b21, because knock-in mice with a mutation in the integrin-binding site of QBRICK developed normally and do not exhibit any defects in integrin \u03b18\u03b21 binding. Instead, the loss of QBRICK significantly diminished the expression of nephronectin, an integrin \u03b18\u03b21 ligand necessary for renal development. In vivo, nephronectin associated with QBRICK and localized at the sublamina densa region, where QBRICK was also located. Collectively, these findings indicate that QBRICK facilitates the integrin \u03b18\u03b21\u2013dependent interactions of cells with basement membranes by regulating the basement membrane assembly of nephronectin and explain why renal defects occur in Fraser syndrome.<\/jats:p>","DOI":"10.1083\/jcb.201203065","type":"journal-article","created":{"date-parts":[[2012,5,21]],"date-time":"2012-05-21T22:50:41Z","timestamp":1337640641000},"page":"677-689","update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":53,"title":["Basement membrane assembly of the integrin \u03b18\u03b21 ligand nephronectin requires Fraser syndrome\u2013associated proteins"],"prefix":"10.1083","volume":"197","author":[{"given":"Daiji","family":"Kiyozumi","sequence":"first","affiliation":[{"name":"Laboratory of Extracellular Matrix Biochemistry, Institute for Protein Research, Osaka University, Suita, Osaka 565-0871, Japan 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Makiko","family":"Takeichi","sequence":"additional","affiliation":[{"name":"Laboratory of Extracellular Matrix Biochemistry, Institute for Protein Research, Osaka University, Suita, Osaka 565-0871, Japan 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Itsuko","family":"Nakano","sequence":"additional","affiliation":[{"name":"Laboratory of Extracellular Matrix Biochemistry, Institute for Protein Research, Osaka University, Suita, Osaka 565-0871, Japan 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuya","family":"Sato","sequence":"additional","affiliation":[{"name":"Laboratory of Extracellular Matrix Biochemistry, Institute for Protein Research, Osaka University, Suita, Osaka 565-0871, Japan 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomohiko","family":"Fukuda","sequence":"additional","affiliation":[{"name":"Sekiguchi Biomatrix Signaling Project, Japan Science and Technology Agency, Aichi Medical University, Nagakute, Aichi 480-1195, Japan 2"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kiyotoshi","family":"Sekiguchi","sequence":"additional","affiliation":[{"name":"Laboratory of Extracellular Matrix Biochemistry, Institute for Protein Research, Osaka University, Suita, Osaka 565-0871, Japan 1"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2012,5,21]]},"reference":[{"key":"2023072302310644500_bib1","first-page":"36","article-title":"Image Processing with ImageJ","volume":"11","author":"Abramoff","year":"2004","journal-title":"Biophotonics International."},{"key":"2023072302310644500_bib2","doi-asserted-by":"publisher","first-page":"414","DOI":"10.1016\/j.ajhg.2009.08.010","article-title":"FREM1 mutations cause bifid nose, renal agenesis, and anorectal malformations syndrome","volume":"85","author":"Alazami","year":"2009","journal-title":"Am. 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