{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,14]],"date-time":"2025-10-14T00:54:09Z","timestamp":1760403249668,"version":"build-2065373602"},"reference-count":52,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2022,9,20]],"date-time":"2022-09-20T00:00:00Z","timestamp":1663632000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["anatomypubs.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Developmental Dynamics"],"published-print":{"date-parts":[[2023,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Although splicing is an integral part of the expression of many genes in our body, genetic syndromes with spliceosomal defects affect only specific tissues. To help understand the mechanism, we investigated the expression pattern of a core protein of the major spliceosome, SmB\/B\u2032 (Small Nuclear Ribonucleoprotein Polypeptides B\/B\u2032), which is encoded by <jats:italic>SNRPB<\/jats:italic>. Loss\u2010of\u2010function mutations of <jats:italic>SNRPB<\/jats:italic> in humans cause cerebro\u2010costo\u2010mandibular syndrome (CCMS) characterized by rib gaps, micrognathia, cleft palate, and scoliosis. Our expression analysis focused on the affected structures as well as non\u2010affected tissues, using chick and mouse embryos as model animals.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Embryos at young stages (gastrula) showed ubiquitous expression of SmB\/B\u2032. However, the level and pattern of expression became tissue\u2010specific as differentiation proceeded. The regions relating to CCMS phenotypes such as cartilages of ribs and vertebrae and palatal mesenchyme express SmB\/B\u2032 in the nucleus sporadically. However, cartilages that are not affected in CCMS also showed similar expressions. Another spliceosomal gene, <jats:italic>SNRNP200<\/jats:italic>, which mutations cause retinitis pigmentosa, was also prominently expressed in cartilages in addition to the retina.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The expression of SmB\/B\u2032 is spatiotemporally regulated during embryogenesis despite the ubiquitous requirement of the spliceosome, however, the expression pattern is not strictly correlated with the phenotype presentation.<\/jats:p><\/jats:sec>","DOI":"10.1002\/dvdy.537","type":"journal-article","created":{"date-parts":[[2022,9,4]],"date-time":"2022-09-04T22:29:48Z","timestamp":1662330588000},"page":"276-293","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Non\u2010ubiquitous expression of core spliceosomal protein SmB\/B\u2032 in chick and mouse embryos"],"prefix":"10.1002","volume":"252","author":[{"given":"Benedict R. 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