McLeod Cathy M, Lanthier Hannah G, Nitschke Garrett R, Bhandari Samjhana, Humphries Angel C, Garrett Andrew M
Department of Pharmacology, Wayne State University School of Medicine, Detroit, MI, USA.
Department of Ophthalmology, Visual, and Anatomical Sciences, Wayne State University School of Medicine, Detroit, MI, USA.
Mol Neurobiol. 2025 Jul 31. doi: 10.1007/s12035-025-05239-z.
Developmental apoptosis is an important mechanism for the formation of functional neural circuits. Distinct neuronal subtypes undergo apoptosis to a greater or lesser extent during development, although how this is regulated at the cell type level is unknown. The clustered protocadherins (cPcdhs) are ~ 60 homophilic cell adhesion molecules expressed from three contiguous gene clusters, which together encode the α-, β-, and γ-Pcdh families. Only one cPcdh isoform, γC4, is essential for survival in the mouse, given its role in attenuating the extent of developmental neuronal apoptosis. However, there is also evidence that other isoforms contribute to neuronal survival. Here, we focused on amacrine cell types in the mouse retina, using a series of genetic models to ascertain that γC4 alone accounts for the pro-survival function of the γ-Pcdhs, and that neuronal subtype dependence on γ-Pcdhs for survival correlates with expression of this single isoform. To test which domains of the protein were essential for this function, we employed a rescue approach with in vitro live cell imaging, finding that the unique variable cytoplasmic domain of γC4-not its adhesive extracellular cadherin repeats-is necessary and sufficient to promote neuronal survival.
发育性细胞凋亡是功能性神经回路形成的重要机制。在发育过程中,不同的神经元亚型或多或少都会经历细胞凋亡,尽管在细胞类型水平上其调控方式尚不清楚。成簇原钙黏蛋白(cPcdhs)是由三个相邻基因簇表达的约60种嗜同性细胞黏附分子,它们共同编码α-、β-和γ-Pcdh家族。鉴于γC4在减轻发育性神经元凋亡程度方面的作用,只有一种cPcdh亚型γC4对小鼠的存活至关重要。然而,也有证据表明其他亚型对神经元存活有贡献。在这里,我们聚焦于小鼠视网膜中的无长突细胞类型,使用一系列遗传模型来确定仅γC4就解释了γ-Pcdhs的促存活功能,并且神经元亚型对γ-Pcdhs存活的依赖性与这种单一亚型的表达相关。为了测试该蛋白的哪些结构域对这一功能至关重要,我们采用了体外活细胞成像的拯救方法,发现γC4独特的可变胞质结构域——而非其黏附性的细胞外钙黏蛋白重复序列——对于促进神经元存活是必要且充分的。