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牙缺失的基因型和表型相关性:综合征和非综合征形式中 WNT10A 和 EDA 突变的启示。

Genotypic and phenotypic correlations in tooth agenesis: insights from WNT10A and EDA mutations in syndromic and non-syndromic forms.

机构信息

Department of Stomatology, Nanfang Hospital, Southern Medical University, Guangzhou, 510515, China.

出版信息

Hum Genet. 2024 Nov;143(11):1253-1264. doi: 10.1007/s00439-024-02705-x. Epub 2024 Sep 25.

Abstract

Tooth agenesis (TA) occurs when tooth development is disrupted at the initiation stage. It can be classified into non-syndromic and syndromic forms (named NSTA and STA), depending on whether it is accompanied by abnormalities of other organs and systems. Genetic factors play a predominant role in the pathogenesis of tooth agenesis, with dozens of genes implicated in both forms. Several genes have been identified, mutations in which can lead to both forms of TA. Among these, WNT10A and EDA are frequently mutated genes in this context, representing extensively researched and documented genes in human non-syndromic selective agenesis of permanent teeth and their association with ectodermal dysplasia syndromes. In this review, we present an overview of the current knowledge regarding genes associated with NSTA and STA, focusing on the distribution and nature of WNT10A and EDA gene mutations. We also explore how these mutations relate to the condition's both forms, including their association with the number of missing permanent teeth.

摘要

牙齿缺失(TA)是指牙齿在起始阶段发育受到干扰。它可以分为非综合征性和综合征性形式(分别命名为 NSTA 和 STA),这取决于它是否伴有其他器官和系统的异常。遗传因素在牙齿缺失的发病机制中起着主导作用,数十种基因与这两种形式都有关。已经确定了几个基因,这些基因的突变可导致两种形式的 TA。其中,WNT10A 和 EDA 是该背景下经常发生突变的基因,它们是人类非综合征性永久性牙齿选择性缺失及其与外胚层发育不良综合征关联的广泛研究和记录的基因。在这篇综述中,我们介绍了与 NSTA 和 STA 相关基因的最新知识概述,重点介绍了 WNT10A 和 EDA 基因突变的分布和性质。我们还探讨了这些突变与该病症的两种形式的关系,包括它们与缺失的永久性牙齿数量的关联。

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