Biochemical and structural characterization of a family with dentine dysplasia
Biochemical and structural characterization of a family with dentine dysplasia
复制标题
牙本质发育不良家族的生化和结构特征
DOI：
10.2330/joralbiosci1965.32.591
复制
发表时间：
1990
期刊：
影响因子：
--
通讯作者：
H. Aoki
中科院分区：
文献类型：
--
作者：
T. Takagi;S. Sasaki;K. Kashima;S. Shioiri;S. Shioda;Satoshi Nakamura;M. Akao;H. Aoki
牙本质发育不良是一种非常罕见的先天性疾病，是牙本质发育异常的一种特殊表现。一个儿子和一个女儿，他们的母亲已经被诊断为患有这种疾病，进行了详细的病理和生化研究。患者的牙齿表现出所有的临床，放射学和病理特征的根性牙本质发育不良（I型）已被发现有致密的骨质疏松。对牙本质的无机和有机成分进行了生化分析，结果表明：牙本质的无机成分为择优取向的羟基磷灰石多晶。通过XRD估算的Ca/P原子比为1.67，不缺乏Ca，并且与化学计量的羟基磷灰石的理论值很好地一致。大部分有机成分不能被胃蛋白酶消化，牙本质的胶原表型为I型。检查他们的一般情况表明，患者患有骨质疏松症，这表明存在不同类型的牙本质发育不良，与一些一般疾病有关。
Dentine dysplasia has been known as exceedingly rare, congenital disease which cause the specific disformation of dentine. A son and a daughter, whose mother was already diagnosed as having this disease, were studied pathologically and biochemically in detail. Patients with teeth showing all of the clinical, radiographic, and pathological features of radicular dentine dysplasia (type I) have been found to have dense sclerotic bone. Biochemical analyses of the inorganic and organic components of dentine gave the following results: The inorganic component was preferred-oriented hydroxyapatite polycrystal. The Ca/P atomic ratio of 1.67, estimated by XRD, was not deficient in Ca, and agreed well with the theoretical value of stoichiometric hydroxyapatite. Most of the organic component could not be digested by pepsin and the collagen phenotype of the dentine was found to be type I. Examination of their general condition showed the patients to be suffering from osteosclerosis, which suggests the existence of a different type of dentine dysplasia, related to some general disorder.