The fundamentals of WNT10A

IF 2.2 3区生物学 Q4 CELL BIOLOGY

Differentiation Pub Date : 2025-03-01 DOI:10.1016/j.diff.2025.100838

Erica L. Benard , Matthias Hammerschmidt

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引用次数: 0

Abstract

Human wingless-type MMTV integration site family member 10A (WNT10A) is a secreted glycoprotein that is involved in signaling pathways essential to ectodermal organogenesis and tissue regeneration. WNT10A was first linked to human disorders in 2006, demonstrating a WNT10a variant to be associated with cleft lip with/without cleft palate. Numerous publications have since then identified the importance of WNT10A in the development of ectodermal appendages and beyond. In this review, we provide information on the structure of the WNT10A gene and protein, summarize its expression patterns in different animal models and in human, and describe the identified roles in tissue and organ development and repair in the different animal model organisms. We then correlate such identified functions and working mechanisms to the pathophysiology of a spectrum of human diseases and disorders that result from germline loss-of-function mutations in WNT10A, including ectodermal dysplasia (ED) syndromes Odonto-oncho-dermal dysplasia (OODD), Schöpf–Schulz–Passarge syndrome (SSPS), and selective tooth agenesis, as well as pathological conditions like fibrosis and carcinogenesis that can be correlated with increased WNT10A activity (Section 5).

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WNT10A 的基本原理。

人类无翼型MMTV整合位点家族成员10A （WNT10A）是一种分泌糖蛋白，参与外胚层器官发生和组织再生所必需的信号通路。WNT10A于2006年首次与人类疾病联系起来，证明WNT10A变体与伴有/不伴有腭裂的唇裂有关。从那时起，许多出版物已经确定了WNT10A在外胚层附属物及其他发育中的重要性。本文综述了WNT10A基因和蛋白的结构，总结了其在不同动物模型和人类中的表达模式，并描述了其在不同动物模型生物中组织和器官发育和修复中的作用。然后，我们将这些已确定的功能和工作机制与WNT10A种系功能丧失突变引起的一系列人类疾病和病症的病理生理学联系起来，包括外胚层发育不良（ED）综合征齿-软骨-真皮发育不良（OODD）、Schöpf-Schulz-Passarge综合征（SSPS）和选择性牙齿发育，以及与WNT10A活性增加相关的纤维化和癌变等病理状况（第5节）。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Differentiation 生物-发育生物学

CiteScore

4.10

自引率

3.40%

发文量

审稿时长

51 days

期刊介绍： Differentiation is a multidisciplinary journal dealing with topics relating to cell differentiation, development, cellular structure and function, and cancer. Differentiation of eukaryotes at the molecular level and the use of transgenic and targeted mutagenesis approaches to problems of differentiation are of particular interest to the journal. The journal will publish full-length articles containing original work in any of these areas. We will also publish reviews and commentaries on topics of current interest. The principal subject areas the journal covers are: • embryonic patterning and organogenesis • human development and congenital malformation • mechanisms of cell lineage commitment • tissue homeostasis and oncogenic transformation • establishment of cellular polarity • stem cell differentiation • cell reprogramming mechanisms • stability of the differentiated state • cell and tissue interactions in vivo and in vitro • signal transduction pathways in development and differentiation • carcinogenesis and cancer • mechanisms involved in cell growth and division especially relating to cancer • differentiation in regeneration and ageing • therapeutic applications of differentiation processes.