The analysis of enamel malformation in type VII collagen knockout mice.

VII型胶原蛋白敲除小鼠牙釉质畸形分析。

• 批准号: 24890008
• 负责人: ASAKA Takuya
• 金额: $ 1.91万
• 依托单位: Hokkaido University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Research Activity Start-up
• 财政年份: 2012
• 资助国家: 日本
• 起止时间: 2012-08-31 至 2014-03-31
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-24890008/
• 关键词: ７型コラーゲン; エナメル芽細胞; トームス突起; エナメル小柱

Recessive dystrophic epidermolysis bullosa (RDEB) is caused by mutations in the gene encoding type VII collagen (COL7), a major component of anchoring fibrils in the epidermal basement membrane zone. We examined the tooth enamel structure of an RDEB patient by scanning electron microscopy. It showed irregular enamel prisms, indicating structural enamel defects. The enamel calcification and chemical composition of Col7a1-/- mice were similar to those of the wild type. However, transverse sections of teeth from the Col7a1-/- mice showed irregular enamel prisms, which were also observed in the RDEB patient.Furthermore, the Col7a1-/- mice teeth had poorly differentiated ameloblasts, lacking normal enamel protein-secreting Tomes processes, and showed reduced mRNA expression of amelogenin and other enamel-related molecules. These findings suggest that COL7 regulates ameloblast differentiation and is essential for the formation of Tomes processes.

隐性营养不良大疱性表皮松解症（RDEB）是由编码VII型胶原蛋白（COL7）的基因突变引起的，COL7是表皮基底膜区锚定原纤维的主要成分。我们用扫描电子显微镜检查了一例RDEB患者的牙釉质结构。牙釉质呈不规则棱柱状，提示结构性牙釉质缺陷。Col7a1-/-小鼠的牙釉质钙化和化学成分与野生型相似。然而，Col7a1-/-小鼠的牙齿横切面显示不规则的牙釉质棱柱，这在RDEB患者中也观察到。此外，Col7a1-/-小鼠牙齿的成釉细胞分化较差，缺乏正常的牙釉质蛋白分泌过程，并且成釉原蛋白和其他牙釉质相关分子的mRNA表达减少。这些发现表明，COL7调节成釉细胞的分化，对Tomes过程的形成至关重要。

项目成果

EBにおける歯牙異常の原因と口腔対策

EB牙齿异常的原因及口腔对策

• 发表时间: 2013
• 作者: A. Saeki;T. Segawa;T. Abe;M. Sugiyama;T. Arimoto;H. Hara;A.Hasebe;M. Ohtani;N. Tanizume;M. Ohuchi;H. Kataoka;M. Kawanami;A. Yokoyama and K. Shibata.;浅香卓哉
• 通讯作者: 浅香卓哉

Type VII collagen deficiency causesdefective tooth enamel formation due to poor differentiation of ameloblasts

由于成釉细胞分化不良，VII 型胶原蛋白缺乏会导致牙釉质形成缺陷

• DOI: 10.1016/j.ajpath.2012.07.018
• 发表时间: 2012
• 期刊: Am J Pathol
• 影响因子: 6
• 作者: Umemoto H;Akiyama M;Domon T;Nomura T;Shinkuma S;Ito K;Asaka T;Sawamura D;Uitto J;Uo M;Kitagawa Y;Shimizu H
• 通讯作者: Shimizu H