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《Journal of Peking University(Health Sciences)》 2007-01
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Research advances in tooth agenesis[WT5”BZ]

FENG Hai-lang△, ZHANG Xiao-xia, WU Hua ( Department of Prosthodontic, Peking University School & Hospital of Stomatology, Beijing 100081, China)  
Tooth agenesis constitutes one of the most common developmental anomalies in man. Oligodontia is defined as congenital absence of six or more teeth. Based on the studies of our team in cooperation with Peking University Center for Human Disease Genomics in the past five years, this article reviews the current research progress in clinical phenotypes and case collection, epidemiological investigation and etiological genetic studies of oligodontia. The symptoms of oligodontia were classified into syndromic and non-syndromic according to the occurrence of tooth agenesis with or without systemic developmental defects. As for the advancement of theories and techniques of molecular genetics, a number of gene mutations have been identified to be the direct etiological factors causing some specified diseases, especially those with developmental defects. Here, this article summarized the outcomes of molecular genetic study of some cases we collected. Of the systemic oligondontia patients, a new four-base-deletion mutation in PITX2 was identified in a large kindred with typical symptoms of Rieger Syndrome; four different gene mutations in ED1 casing X-linked hypohidrotic ectodermal displasia were found in five nucleus families. Compared with the former, non-syndromic oligodontia has more genetic heterogeneity rather than some specific virulence gene. PAX9 and MSX1 are the identified genes associated with family tooth agenesis without systemic syndrome. Also, in our research, three gene mutations in CBFA1 were detected in four cleidocranial dysplasia families, which is a systemic developmental disease including the symptoms of tooth eruption abnormality and accessory teeth.
【Fund】: 教育部教育振兴行动计划特殊专项(九八五)工程(2001-10);; “十五211”工程项目子课题;; 北京市自然科学基金项目(7063099)~~
【CateGory Index】: R781
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【Citations】
Chinese Journal Full-text Database 1 Hits
1 WANG Ying 1* , ZHAO Hongshan 2* , ZHANG Xiaoxia 1, FENG Hailan 1△ (1. Department of Prosthodontics, Peking University School of Stomatology, Beijing 100081, China ;2. Department of Immunology, Peking University School of Basic Medical Science, Peking University Center for Human Disease Genomics);Mutation detection in ED1 gene in hypohidrotic ectodermal dysplasia (HED) families[J];Journal of Peking University(Health Sciences);2003-04
【Co-citations】
Chinese Journal Full-text Database 10 Hits
1 WANG Ying 1* , ZHAO Hongshan 2* , ZHANG Xiaoxia 1, FENG Hailan 1△ (1. Department of Prosthodontics, Peking University School of Stomatology, Beijing 100081, China ;2. Department of Immunology, Peking University School of Basic Medical Science, Peking University Center for Human Disease Genomics);Mutation detection in ED1 gene in hypohidrotic ectodermal dysplasia (HED) families[J];Journal of Peking University(Health Sciences);2003-04
2 CUI Juan-juan, LI Wu-li, MEI Ling-xuan. (Dept. of En- dodontics and Operative Dentisry, Hospital of Stomatology, Anhui Medical University, Hefei 230032, China);The study on the PAX 9 related with oligodontia[J];International Journal of Stomatology;2008-01
3 DU Rui-tian, LI Yan. (Dept. of Prosthodontics, Guanghua School of Stomatology, Sun Yat-Sen University, Guangzhou 510055, China);Clinical features and prosthodontics of hypohydrotic ectodermal dysplasia[J];International Journal of Stomatology;2008-01
4 FANG Jing-xian,SONG Guang-tai,YE Xiao-qian.(Dept. of Pediatric Dentistry,College of Stomatology,Wuhan University,Wuhan 430079,China; Key Labo-ratory of Oral Biomedical Engineering of Ministry of Education,Wuhan University,Wuhan 430079,China);Muscle segment homeobox gene-1 and non-syndromic hypodontia[J];International Journal of Stomatology;2009-02
5 CHANG Qing, KONG Xiangdong, SHI Huirong, GUO Hongjun, ZHANG Yingying Department of Gynecology and Obstetrics, the First Affiliated Hospital, Zhengzhou University, Zhengzhou450052;Prenatal diagnosis in one family with X-linked hypohidrotic ectodermal dysplasia[J];Journal of Zhengzhou University(Medical Sciences);2008-01
6 YANG Chang-hu, JIN Fang, GUO Dan, QI Rong-jin, ZHOU Xue-jun. (Department of Stomatology, Zhen'an Hospital of Traditional Chinese Medicine, Zhen'an 711500 ,China);Retrospective study of 62 malocclusion patients with congenitally missing teeth[J];Stomatology;2007-01
7 PAN Yong-chu,MA Jun-qing,WANG Lin,ZHANG Wei-bing,GU Ning,HUANG You,CHEN Li-ming.(The Research Institute of Stomatology,Nanjing Medical University,Nanjing 210029,China);Dento-facial morphology study of female children with congenital absence of mandibular incisor[J];Stomatology;2008-03
8 LIU li1,DUAN Yin-zhong1,WANG Hong-lei2 (1.Department of Orthodontics,Stomatological College,the Fourth Military Medical University,Xi an 710032,Shaanxi,China;2.322 Hospital of Chinese PLA);Orthodontic treatment for patients with congenital missing lower incisors[J];Chinese Journal of Aesthetic Medicine;2008-02
9 WU Qi-zhu,Aolibu,HUANG Ya-feng,BAI Hai-hua(Inner Mongolia University for Nationalities,Tongliao 028041,China);The Clinical Analysis of A Large Rare Mongolian Family with Ectodermal Dysplasia[J];Journal of Inner Mongolia University for Nationalities(Natural Sciences,Quarterly);2005-03
10 JIANG Lan1,LI Yong2,LAI Wen-li1.(1.Dept.of Orthodontics,West China College of Stomatology,Sichuan University,Chengdu 610041,China;2.Dept.of Prosthodontics,West China College of Stomatology,Sichuan University,Chengdu 610041,China);Cleidocranial dysplasia:A case report[J];West China Journal of Stomatology;2009-04
【Co-references】
Chinese Journal Full-text Database 1 Hits
1 LEI Ke, HE Xiang-yi. (Dept. of Prosthodontics, College of Stomatology, Lanzhou University, Lanzhou 730000, China);Research progress in the genes and proteins of hypohidrosis ectodermal dysplasia[J];International Journal of Stomatology;2009-01
【Secondary Citations】
Chinese Journal Full-text Database 1 Hits
1 LI Rui, LIU Hong-chen, HUANG Xu-ming (Department of stomatology, General hospital of PLA, BeiJing 100853, China);Cleidocranial dysplasia (CCD)-a case report[J];Chinese Journal of Prosthodontics;2003-04
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