Department of Ophthalmology, The Second Hospital of Jilin University, Changchun, 130000, China.
College of Basic Medical Sciences, Jilin University, Changchun, 130000, China.
Cell Commun Signal. 2024 May 27;22(1):290. doi: 10.1186/s12964-024-01673-z.
The Crumbs protein (CRB) family plays a crucial role in maintaining the apical-basal polarity and integrity of embryonic epithelia. The family comprises different isoforms in different animals and possesses diverse structural, localization, and functional characteristics. Mutations in the human CRB1 or CRB2 gene may lead to a broad spectrum of retinal dystrophies. Various CRB-associated experimental models have recently provided mechanistic insights into human CRB-associated retinopathies. The knowledge obtained from these models corroborates the importance of CRB in retinal development and maintenance. Therefore, complete elucidation of these models can provide excellent therapeutic prospects for human CRB-associated retinopathies. In this review, we summarize the current animal models and human-derived models of different CRB family members and describe the main characteristics of their retinal phenotypes.
CRB 蛋白（CRB）家族在维持胚胎上皮的顶端-基底极性和完整性方面发挥着关键作用。该家族在不同的动物中包含不同的同工型，具有不同的结构、定位和功能特征。人类 CRB1 或 CRB2 基因突变可能导致广泛的视网膜营养不良。最近，各种与 CRB 相关的实验模型为人类 CRB 相关的视网膜病变提供了机制上的见解。这些模型获得的知识证实了 CRB 在视网膜发育和维持中的重要性。因此，对这些模型的全面阐述可为人类 CRB 相关的视网膜病变提供极好的治疗前景。在这篇综述中，我们总结了不同 CRB 家族成员的现有动物模型和人类衍生模型，并描述了它们的视网膜表型的主要特征。
