摘要
Gp280/内在因子-维生素B12受体/ CUBN是一种大的内吞性受体,在维生素B12稳态、蛋白质或毒性物质(包括白蛋白、维生素d结合蛋白或镉)的肾重吸收中发挥多种作用。Cubilin是由8个表皮生长因子(EGF)样重复和27个CUB(定义为补体C1r/C1s, Uegf, BMP1)结构域组成的外周膜蛋白。这种结构独特的蛋白质至少与两种分子伙伴相互作用,无羊膜(AMN)和Lrp2/Megalin。AMN参与了Cubilin的质膜转运,而Lrp2对Cubilin及其配体的有效内化至关重要。从Cubn缺乏或人类疾病的动物模型中收集的观察结果表明了该蛋白的重要性。本文综述了近年来有关铜胆素及其在肾和肠生物学中的应用,并对基础研究和医学工作者进行了综述。我们还讨论了Cubilin在胚胎发育过程中作为Fgf8信号调节因子的作用,并提出Cubilin-Fgf8相互作用可能与人类病理相关,包括癌症进展、心脏或神经管缺陷。最后,我们提供了一些实验元素,提示Cubilin生理学的某些方面可能与药物设计有关。
关键词: 维生素B12
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