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学科主题: 口腔医学
题名:
Genetic Etiology and Dental Pulp Cell Deficiency of Hypophosphatasia
作者: Liu, H.1,2; Li, J.1,2; Lei, H.1,2; Zhu, T.1,2; Gan, Y.3,4; Ge, L.1,2
关键词: hypophosphatasia ; tissue-non-specific alkaline phosphatase ; gene mutation ; dental pulp cells
刊名: JOURNAL OF DENTAL RESEARCH
发表日期: 2010-12-01
DOI: 10.1177/0022034510379017
卷: 89, 期:12, 页:1373-1377
收录类别: SCI
文章类型: Article
WOS标题词: Science & Technology
类目[WOS]: Dentistry, Oral Surgery & Medicine
研究领域[WOS]: Dentistry, Oral Surgery & Medicine
关键词[WOS]: NONSPECIFIC ALKALINE-PHOSPHATASE ; STRUCTURAL EVIDENCE ; MINERALIZATION ; MUTATIONS ; CULTURES ; TEETH
英文摘要:

Hypophosphatasia is caused by mutations of the tissue-non-specific alkaline phosphatase (TNSALP) gene with deficiency of dentin structure. The aim of this study was to examine whether TNSALP mutation in dental pulp cells contributes to dentin dysplasia in hypophosphatasia. Mutation analysis showed that compound heterozygous mutations of TNSALP were identified in three hypophosphatasia patients, including 3 novel mutation sites. Exfoliated teeth from the patients showed abnormal dentin mineralization and loss of cementum, as assessed by ground sections and scanning electron microscope analysis. Dental pulp cells isolated from one of the patients showed a significantly reduced TNSALP activity and mineralization capacity when compared with those in dental pulp cells from the unaffected individuals. Our results suggested that dentin dysplasia in hypophosphatasia may be associated with the decreased mineralization ability of dental pulp cells.

语种: 英语
所属项目编号: 30772419
项目资助者: National Natural Science Foundation of China
WOS记录号: WOS:000284531100006
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/64036
Appears in Collections:北京大学口腔医学院_儿童口腔科_期刊论文

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作者单位: 1.Peking Univ, Dept Pediat Dent, Sch Stomatol, Beijing 100871, Peoples R China
2.Peking Univ, Dept Pediat Dent, Hosp Stomatol, Beijing 100871, Peoples R China
3.Peking Univ, Cent Lab, Hosp Stomatol, Beijing 100871, Peoples R China
4.Peking Univ, Cent Lab, Sch Stomatol, Beijing 100871, Peoples R China

Recommended Citation:
Liu, H.,Li, J.,Lei, H.,et al. Genetic Etiology and Dental Pulp Cell Deficiency of Hypophosphatasia[J]. JOURNAL OF DENTAL RESEARCH,2010,89(12):1373-1377.
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