Dentin noncollagenous matrix proteins in familial hypophosphatemic rickets.
Dentin noncollagenous matrix proteins in familial hypophosphatemic rickets.
复制标题
DOI：
10.1159/000151382
复制
发表时间：
2009
期刊：
影响因子：
--
通讯作者：
Chaussain-Miller C
中科院分区：
文献类型：
--
作者：
Gaucher C;Boukpessi T;Septier D;Jehan F;Rowe PS;Garabédian M;Goldberg M;Chaussain-Miller C
Familial hypophosphatemic rickets is transmitted in most cases as an X-linked dominant trait and results from the mutation of the PHEX gene predominantly expressed in osteoblast and odontoblast. Patients with rickets have been reported to display important dentin defects. Our purpose was to explore the structure, composition and distribution of noncollagenous proteins (NCPs) of hypophosphatemic dentin. We collected teeth from 10 hypophosphatemic patients whose mineralization occurred either in a hypophosphatemic environment or in a corrected phosphate and vitamin environment. Teeth were examined by scanning electron microscopy, immunohistochemistry and Western blot analysis. An abnormal distribution (accumulation in interglobular spaces) and cleavage of the NCPs and particularly of matrix extracellular phosphoglycoprotein were observed in deciduous dentin. In contrast, it was close to normal in permanent dentin mineralized under corrected conditions. In conclusion, dentin mineralization in a corrected phosphate and vitamin D environment compensates the adverse effect of PHEX mutation.
登录
查看更多内容
影响因子：
4.1
作者：
Boileau, G;Tenenhouse, HS;Crine, P
通讯作者：
Crine, P
影响因子：
2.9
作者：
Goldberg, M;Septier, D;Menashi, S
通讯作者：
Menashi, S
影响因子：
30.8
作者：
Lorenz-Depiereux, Bettina;Bastepe, Murat;Strom, Tim M.
通讯作者：
Strom, Tim M.
影响因子：
2.9
作者：
Fisher, LW;Fedarko, NS
通讯作者：
Fedarko, NS
影响因子：
5.1
作者：
Chaussain-Miller, C;Sinding, C;Garabédian, M
通讯作者：
Garabédian, M