The nuclear lamina is a multi-protein lattice composed of A- and B-type lamins and their associated proteins. This protein lattice associates with heterochromatin and integral inner nuclear membrane proteins, providing links among the genome, nucleoskeleton, and cytoskeleton. In the 1990s, mutations in EMD and LMNA were linked to Emery-Dreifuss muscular dystrophy. Since then, the number of diseases attributed to nuclear lamina defects, including laminopathies and other disorders, has increased to include more than 20 distinct genetic syndromes. Studies of patients and mouse genetic models have pointed to important roles for lamins and their associated proteins in the function of gastrointestinal organs, including liver and pancreas. We review the interactions and functions of the lamina in relation to the nuclear envelope and genome, the ways in which its dysfunction is thought to contribute to human disease, and possible avenues for targeted therapies.

核层是由A型和B型lamin及其相关蛋白组成的多蛋白晶格。该蛋白晶格与异染色质和完整的内核膜蛋白缔合，提供了基因组，核骨架和细胞骨架之间的联系。在1990年代，EMD和LMNA发生了突变与Emery-Dreifuss肌营养不良有关。从那时起，归因于核椎板缺损的疾病数量（包括椎间盘突出症和其他疾病）增加到包括20多种不同的遗传综合征。对患者和小鼠遗传模型的研究指出了lamin及其相关蛋白在胃肠器官（包括肝和胰腺）功能中的重要作用。我们审查了与核膜和基因组有关的层板的相互作用和功能，认为其功能障碍导致人类疾病的方式以及针对性疗法的可能途径。