{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,4]],"date-time":"2026-03-04T14:36:25Z","timestamp":1772634985699,"version":"3.50.1"},"reference-count":30,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2023,4,5]],"date-time":"2023-04-05T00:00:00Z","timestamp":1680652800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Shriners Children\u2019s","award":["SFB 829-B2"],"award-info":[{"award-number":["SFB 829-B2"]}]},{"name":"Shriners Children\u2019s","award":["FOR2722-407164210"],"award-info":[{"award-number":["FOR2722-407164210"]}]},{"name":"German Research Foundation","award":["SFB 829-B2"],"award-info":[{"award-number":["SFB 829-B2"]}]},{"name":"German Research Foundation","award":["FOR2722-407164210"],"award-info":[{"award-number":["FOR2722-407164210"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>AMACO (VWA2 protein), secreted by epithelial cells, is strongly expressed at basement membranes when budding or invagination occurs in embryos. In skin, AMACO associates with proteins of the Fraser complex, which form anchoring cords. These, during development, temporally stabilize the dermal\u2013epidermal junction, pending the formation of collagen VII-containing anchoring fibrils. Fraser syndrome in humans results if any of the core members of the Fraser complex (Fras1, Frem1, Frem2) are mutated. Fraser syndrome is characterized by subepidermal blistering, cryptophthalmos, and syndactyly. In an attempt to determine AMACO function, we generated and characterized AMACO-deficient mice. In contrast to Fraser complex mutant mice, AMACO-deficient animals lack an obvious phenotype. The mutually interdependent basement membrane deposition of the Fraser complex proteins, and the formation of anchoring cords, are not affected. Furthermore, hair follicle development in newborn AMACO-deficient mice showed no gross aberration. Surprisingly, it appears that, while AMACO is a component of the anchoring cords, it is not essential for their formation or function.<\/jats:p>","DOI":"10.3390\/ijms24076782","type":"journal-article","created":{"date-parts":[[2023,4,5]],"date-time":"2023-04-05T05:42:44Z","timestamp":1680673364000},"page":"6782","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["The Fraser Complex Proteins (Frem1, Frem2, and Fras1) Can Form Anchoring Cords in the Absence of AMACO at the Dermal\u2013Epidermal Junction of Mouse Skin"],"prefix":"10.3390","volume":"24","author":[{"given":"Temitope","family":"Esho","sequence":"first","affiliation":[{"name":"Center for Biochemistry, Medical Faculty, University of Cologne, 50931 Cologne, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Birgit","family":"Kobbe","sequence":"additional","affiliation":[{"name":"Center for Biochemistry, Medical Faculty, University of Cologne, 50931 Cologne, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6447-8041","authenticated-orcid":false,"given":"Sara","family":"Tufa","sequence":"additional","affiliation":[{"name":"Micro-Imaging Center, Shriners Children\u2019s, Portland, OR 97239, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Douglas","family":"Keene","sequence":"additional","affiliation":[{"name":"Micro-Imaging Center, Shriners Children\u2019s, Portland, OR 97239, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mats","family":"Paulsson","sequence":"additional","affiliation":[{"name":"Center for Biochemistry, Medical Faculty, University of Cologne, 50931 Cologne, Germany"},{"name":"Center for Molecular Medicine Cologne, 50931 Cologne, Germany"},{"name":"Cologne Center for Musculoskeletal Biomechanics, 50931 Cologne, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raimund","family":"Wagener","sequence":"additional","affiliation":[{"name":"Center for Biochemistry, Medical Faculty, University of Cologne, 50931 Cologne, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,4,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"50240","DOI":"10.1074\/jbc.M307794200","article-title":"Identification and characterization of AMACO, a new member of the von Willebrand factor A-like domain protein superfamily with a regulated expression in the kidney","volume":"278","author":"Sengle","year":"2003","journal-title":"J. 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