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首页> 外文期刊>Molecular and Cellular Biology >Early Postnatal Death and Motor Disorders in Mice Congenitally Deficient in Calnexin Expression
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Early Postnatal Death and Motor Disorders in Mice Congenitally Deficient in Calnexin Expression

机译：先天性钙合蛋白表达不足的小鼠的早期产后死亡和运动障碍

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Calnexin is a ubiquitously expressed type I membrane protein which is exclusively localized in the endoplasmic reticulum (ER). In mammalian cells, calnexin functions as a chaperone molecule and plays a key role in glycoprotein folding and quality control within the ER by interacting with folding intermediates via their monoglucosylated glycans. In order to gain more insight into the physiological roles of calnexin, we have generated calnexin gene-deficient mice. Despite its profound involvement in protein folding, calnexin is not essential for mammalian-cell viability in vivo: calnexin gene knockout mice were carried to full term, although 50% died within 48 h and the majority of the remaining mice had to be sacrificed within 4 weeks, with only a very few mice surviving to 3 months. Calnexin gene-deficient mice were smaller than their littermates and showed very obvious motor disorders, associated with a dramatic loss of large myelinated nerve fibers. Thus, the critical contribution of calnexin to mammalian physiology is tissue specific.
机译：钙结合蛋白是普遍表达的I型膜蛋白，仅定位在内质网（ER）中。在哺乳动物细胞中，钙调蛋白起分子伴侣分子的作用，并通过其单糖基化聚糖与折叠中间体相互作用，从而在ER内糖蛋白折叠和质量控制中发挥关键作用。为了深入了解钙调蛋白的生理作用，我们制备了钙调蛋白基因缺陷小鼠。尽管钙调蛋白在蛋白质折叠中起着重要作用，但对于哺乳动物细胞在体内的生存力并不是必不可少的：钙调蛋白基因敲除小鼠已经足月了，尽管50％的小鼠在48小时内死亡，其余大多数小鼠在4h内被处死。周，只有极少数小鼠存活到3个月。钙结合蛋白基因缺陷的小鼠比同窝小鼠小，并且表现出非常明显的运动障碍，这与大型有髓神经纤维的大量丢失有关。因此，钙调蛋白对哺乳动物生理的关键贡献是组织特异性的。

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