喷发初始破坏机制:一个多维框架新模型。

IF 5 2区 医学 Q1 Dentistry
Xing Hu, Anjie Guo, Yanling Xie, Shuixue Mo
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引用次数: 0

摘要

原发性出牙失败(PFE)是一种罕见的常染色体显性遗传病,主要由受损的牙齿出牙机制定义。它的特征是单侧或双侧在后区开放咬合和不完整或没有牙齿萌出。尽管如此,其病因仍不清楚。目前的研究表明,PFE可能与几种基因的突变直接相关,包括甲状旁腺激素受体1 (PTH1R)、赖氨酸(K)特异性甲基转移酶2c (KMT2C)和跨膜蛋白119 (TMEM119)。尽管对PFE遗传学的研究越来越多,但没有一个统一的概念框架来整合已知的遗传信息。这篇综述旨在解决这一差距,并介绍一种新的致病模型。该模型假设,正常的牙齿萌牙依赖于“促萌轴”和“抗萌轴”之间的精确平衡,而牙毛囊(DF)是这一机制的中心调节枢纽。本研究将对pfe相关基因进行系统分类,并将其致病机制分为“动力不足”和“过度抵抗”。在此基础上,本文将深入分析关键信号通路(如PTH、Wnt、TGF-β、RANKL/OPG)的串扰,探讨遗传影响和时间因素在阐明表型变异性中的复杂性,将现有的分类框架增强为一个动态的、多维的综合网络。并最终从转化医学的角度提出基于致病机制的精确诊断方法和前瞻性治疗靶点。通过构建这一综合病理生理模型,我们旨在为研究PFE的分子机制和临床诊断以及制定新的个体化治疗方案建立一个强大的理论框架。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Mechanisms of primary failure of eruption: a new model of multidimensional framework.

Mechanisms of primary failure of eruption: a new model of multidimensional framework.

Mechanisms of primary failure of eruption: a new model of multidimensional framework.

Mechanisms of primary failure of eruption: a new model of multidimensional framework.

Primary failure of eruption (PFE) is an uncommon autosomal dominant disorder predominantly defined by an impaired tooth eruption mechanism. It is characterized by a unilateral or bilateral open bite in the posterior area and incomplete or absent tooth eruption. Nonetheless, its etiology remains unclear. Current studies indicate that PFE may be directly associated with mutations in several genes, including parathyroid hormone receptor 1 (PTH1R), lysine (K)-specific methyltransferase 2 C (KMT2C), and transmembrane protein 119 (TMEM119). Despite the growing research on PFE genetics, no cohesive conceptual framework exists to integrate the known genetic information. This review seeks to address this gap and introduce a novel pathogenic model. The model posits that the normal eruption of teeth relies on the precise equilibrium between "The Pro-Eruptive Axis" and "The Anti-Resistance Axis," with the Dental Follicle (DF) serving as the central regulatory hub of this mechanism. This research will systematically categorize PFE-related genes and classify their pathogenic mechanisms as "insufficient motivation" or "excessive resistance" in eruption. On this basis, this review will analyze in depth the crosstalk of key signaling pathways (such as PTH, Wnt, TGF-β, and RANKL/OPG), investigate the complexity of genetic influences and temporal factors in elucidating phenotypic variability, enhance the existing classification framework into a dynamic, multi-dimensional comprehensive network, and ultimately suggest precise diagnostic approaches and prospective therapeutic targets based on pathogenic mechanisms from a translational medicine perspective. By constructing this integrated pathophysiological model, we aim to establish a robust theoretical framework for investigating molecular mechanisms and clinical diagnosis and formulating novel individualized treatment options for PFE.

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来源期刊
Progress in Orthodontics
Progress in Orthodontics Dentistry-Orthodontics
CiteScore
7.30
自引率
4.20%
发文量
45
审稿时长
13 weeks
期刊介绍: Progress in Orthodontics is a fully open access, international journal owned by the Italian Society of Orthodontics and published under the brand SpringerOpen. The Society is currently covering all publication costs so there are no article processing charges for authors. It is a premier journal of international scope that fosters orthodontic research, including both basic research and development of innovative clinical techniques, with an emphasis on the following areas: • Mechanisms to improve orthodontics • Clinical studies and control animal studies • Orthodontics and genetics, genomics • Temporomandibular joint (TMJ) control clinical trials • Efficacy of orthodontic appliances and animal models • Systematic reviews and meta analyses • Mechanisms to speed orthodontic treatment Progress in Orthodontics will consider for publication only meritorious and original contributions. These may be: • Original articles reporting the findings of clinical trials, clinically relevant basic scientific investigations, or novel therapeutic or diagnostic systems • Review articles on current topics • Articles on novel techniques and clinical tools • Articles of contemporary interest
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