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Inactivation of Fam20b in the neural crest‐derived mesenchyme of mouse causes multiple craniofacial defects
Author(s) -
Liu Xuenan,
Li Nan,
Zhang Hua,
Liu Jing,
Zhou Nan,
Ran Chunxiao,
Chen Xiaoyan,
Lu Yongbo,
Wang Xiaofang,
Qin Chunlin,
Xiao Jing,
Liu Chao
Publication year - 2018
Publication title -
european journal of oral sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.802
H-Index - 93
eISSN - 1600-0722
pISSN - 0909-8836
DOI - 10.1111/eos.12563
Subject(s) - neural crest , mesenchyme , craniofacial , crest , biology , anatomy , microbiology and biotechnology , genetics , physics , mesenchymal stem cell , embryo , quantum mechanics
The glycosaminoglycan ( GAG ) chains attached to the core proteins of proteoglycans exert multiple roles, such as enriching signal molecules and regulating the binding of ligands to the corresponding receptors. A newly identified kinase – family with sequence similarity 20 member B ( FAM 20B) – is essential for the formation of GAG chains. The FAM 20B protein phosphorylates the initial xylose on the side chain of a serine residue in the protein. Although the GAG chains of proteoglycans are believed to be indispensable during craniofacial development, there are few reports on their exact functions in craniofacial organogenesis. In this study, by mating Wnt1‐cre mice with Fam20b‐floxed mice (Fam20bflox/flox), we created Wnt1‐Cre;Fam20bflox/flox mice in which Fam20b is ablated in the neural crest‐derived mesenchyme. The Wnt1‐Cre;Fam20bflox/flox mice died immediately after birth because of complete cleft palates. In addition to cleft palate, Wnt1‐Cre;Fam20bflox/flox mice also manifested tongue elevation, micrognathia, microcephaly, suture widening, and reduced mineralization in the calvaria, facial bones, and temporomandibular joint. These findings indicate that the proteoglycans formed through the catalysis of FAM 20B are essential for the morphogenesis and mineralization of the craniofacial complex.