Maddala Rupalatha, Allen Ariana, Skiba Nikolai P, Rao Ponugoti Vasantha
Department of Ophthalmology, Duke University School of Medicine, Durham, NC 27710, USA.
Department of Pharmacology and Cancer Biology, Duke University School of Medicine, Durham, NC 27710, USA.
J Cell Sci. 2024 Dec 15;137(24). doi: 10.1242/jcs.262349. Epub 2024 Dec 18.
The transparent ocular lens is essential for vision because it focuses light onto the retina. Despite recognition of the importance of its unique cellular architecture and mechanical properties, the molecular mechanisms governing these attributes remain elusive. This study aims to elucidate the role of ankyrin-B (AnkB, encoded by ANK2), a membrane scaffolding protein, in lens cytoarchitecture, growth and function using a conditional knockout (cKO) mouse model. The AnkB cKO mouse has no defects in lens morphogenesis but exhibited changes that supported a global role for AnkB in maintenance of lens clarity, size, cytoarchitecture, membrane organization and stiffness. Notably, absence of AnkB led to nuclear cataract formation, which was evident from postnatal day 16. AnkB cKO lens fibers exhibit progressive disruption in membrane organization of the spectrin-actin cytoskeleton, cell adhesion proteins and channel proteins; loss and degradation of several membrane proteins [such as NrCAM. N-cadherin (CDH2) and aquaporin-0 (also known as MIP)]; along with a disorganized plasma membrane and impaired membrane interdigitations. Furthermore, absence of AnkB led to decreased lens stiffness. Collectively, these results illustrate the essential role for AnkB in lens architecture, growth and function through its involvement in membrane skeletal and protein organization and stability.
透明的晶状体对于视觉至关重要,因为它将光线聚焦到视网膜上。尽管人们认识到其独特的细胞结构和机械特性的重要性,但控制这些特性的分子机制仍然难以捉摸。本研究旨在使用条件性敲除(cKO)小鼠模型阐明膜支架蛋白锚蛋白B(AnkB,由ANK2编码)在晶状体细胞结构、生长和功能中的作用。AnkB cKO小鼠在晶状体形态发生方面没有缺陷,但表现出一些变化,这些变化支持AnkB在维持晶状体透明度、大小、细胞结构、膜组织和硬度方面发挥全局作用。值得注意的是,AnkB的缺失导致核性白内障形成,这在出生后第16天就很明显。AnkB cKO晶状体纤维在血影蛋白-肌动蛋白细胞骨架、细胞粘附蛋白和通道蛋白的膜组织中表现出渐进性破坏;几种膜蛋白[如NrCAM、N-钙粘蛋白(CDH2)和水通道蛋白-0(也称为MIP)]的丢失和降解;以及质膜紊乱和膜指状交错受损。此外,AnkB的缺失导致晶状体硬度降低。总的来说,这些结果说明了AnkB通过参与膜骨架和蛋白质组织及稳定性在晶状体结构、生长和功能中的重要作用。