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In this study, we generated a BP180 functional\u2013deficient mouse strain by deleting its extracellular domain of humanized NC16A (termed \u0394NC16A mice). We found that BP180 is expressed by bone marrow mesenchymal stem cells (BM-MSC), and its functional deficiency leads to myeloid hyperplasia. Altered granulopoiesis in \u0394NC16A mice is through bone marrow stromal cells evidenced by bone marrow transplantation. Furthermore, the level of G-CSF in bone marrow and circulation were significantly increased in \u0394NC16A mice as compared with wild-type mice. The increased G-CSF was accompanied by an increased activation of the NF-\u03baB signaling pathway in bone marrow and BM-MSC of \u0394NC16A mice. Blockade of G-CSF restored normal granulopoiesis in \u0394NC16A mice. Inhibition of NF-\u03baB signaling pathway significantly reduces the release of G-CSF from \u0394NC16A BM-MSC in vitro and the level of serum G-CSF in \u0394NC16A mice. To our knowledge, these findings provide the first direct evidence that BP180 plays an important role in granulopoiesis through regulating NF-\u03baB signaling pathway in BM-MSC.<\/jats:p>","DOI":"10.4049\/jimmunol.2000784","type":"journal-article","created":{"date-parts":[[2020,9,30]],"date-time":"2020-09-30T21:30:21Z","timestamp":1601501421000},"page":"2786-2794","source":"Crossref","is-referenced-by-count":4,"title":["Non\u2013Cell-Autonomous Activity of the Hemidesmosomal Protein BP180\/Collagen XVII in Granulopoiesis in Humanized NC16A Mice"],"prefix":"10.1093","volume":"205","author":[{"given":"Lin","family":"Lin","sequence":"first","affiliation":[{"name":"Department of Dermatology, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"},{"name":"Oral Biology Program, School of Dentistry, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bin-Jin","family":"Hwang","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"},{"name":"Department of Microbiology and Immunology, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ning","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9185-3548","authenticated-orcid":false,"given":"Paul","family":"Googe","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7308-292X","authenticated-orcid":false,"given":"Luis A","family":"Diaz","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7295-3490","authenticated-orcid":false,"given":"Ed","family":"Miao","sequence":"additional","affiliation":[{"name":"Department of Microbiology and Immunology, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0014-0022","authenticated-orcid":false,"given":"Barbara","family":"Vilen","sequence":"additional","affiliation":[{"name":"Department of Microbiology and Immunology, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nancy E","family":"Thomas","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7846-0395","authenticated-orcid":false,"given":"Jenny","family":"Ting","sequence":"additional","affiliation":[{"name":"Department of Genetics, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhi","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Dermatology, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"},{"name":"Department of Microbiology and Immunology, University of North Carolina at Chapel Hill , Chapel Hill, NC 27599"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2020,11,15]]},"reference":[{"key":"2025030417505715000_r1","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1146\/annurev-pathol-012615-044313","article-title":"Mechanisms of disease: pemphigus and bullous pemphigoid","volume":"11","author":"Hammers","year":"2016","journal-title":"Annu. 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