![\"FAM83H](\"https:\/\/onlinelibrary.wiley.com\/cms\/asset\/135876f0-53b0-4f2d-ba94-25b7674c49c1\/eji70126-gra-0001-m.png\")
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Loss of the casein kinase 1 scaffolding protein FAM83H compromises bone marrow lymphopoiesis, reduces Foxn1<\/i> expression in cortical thymic epithelial cells (TEC), disrupts thymic architecture and TEC identity, and ultimately impairs double-negative thymocyte proliferation and T-cell production.<\/p>\n
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Family of Sequence Similarity 83H (FAM83H\/SACK1H) is primarily expressed in epithelial cells, where it interacts with casein kinase 1 (CK1) and keratins to regulate cytoskeletal organization, cell proliferation, and vesicular trafficking. Mutations in FAM83H<\/i> are known to cause amelogenesis imperfecta, highlighting its critical role in enamel formation. We generated Fam83h<\/i>-deficient mice (Fam83hem2(IMPC)Ccpcz<\/sup>, Fam83h\u2212\/\u2212<\/sup> Loss of the casein kinase 1 scaffolding protein FAM83H compromises bone marrow lymphopoiesis, reduces Foxn1 expression in cortical thymic epithelial cells (TEC), disrupts thymic architecture and TEC identity, and ultimately impairs double-negative thymocyte proliferation and T-cell production. ABSTRACT Family of Sequence Similarity 83H (FAM83H\/SACK1H) is primarily expressed in epithelial cells, where it interacts with casein … Read more<\/a><\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[112,42],"tags":[],"class_list":["post-53828","post","type-post","status-publish","format-standard","hentry","category-european-journal-of-immunology","category-publicaciones"],"_links":{"self":[{"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/posts\/53828","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/comments?post=53828"}],"version-history":[{"count":0,"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/posts\/53828\/revisions"}],"wp:attachment":[{"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/media?parent=53828"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/categories?post=53828"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/tags?post=53828"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}
\n<\/i>) and mice lacking a part of the N-terminal CK1-binding domain (Fam83h\u220687\/\u220687<\/sup>
\n<\/i>). Consistent with other Fam83h<\/i>-deficient models, these mice are subviable, smaller in size, and exhibit a sparse, scruffy coat, scaly skin, general weakness, and hypoactivity. Notably, both strains show impaired lymphoid cell development in early postnatal life. In the thymus, Fam83h<\/i> expression is confined to thymic epithelial cells (TECs), and its deficiency in stromal cells results in disrupted thymic architecture and a severe block in the expansion of double-negative stage 3 (DN3) T cells, ultimately leading to insufficient T cell production. Single-cell transcriptomic analysis reveals that Fam83h\u2212\/\u2212<\/sup>
\n<\/i> cortical TECs (cTECs) express reduced levels of the TEC master regulator Foxn1<\/i>, and its multiple downstream target genes, suggesting a critical role for FAM83H, likely in coordination with CK1, in cTEC maturation.<\/p>","protected":false},"excerpt":{"rendered":"