Michalak Marek, Guo Lei, Robertson Murray, Lozak Mira, Opas Michal
Canadian Institutes of Health Research, Membrane Protein Research Group, Department of Biochemistry, University of Alberta, Edmonton, Alberta, Canada.
Mol Cell Biochem. 2004 Aug;263(1-2):137-42.
Calreticulin is a Ca2+ binding/storage chaperone resident protein of the endoplasmic reticulum. This protein plays a key role in the calreticulin/calnexin cycle and the quality control pathways in the endoplasmic reticulum. Calreticulin deficiency is lethal due to impaired cardiac development. However, over-expression of the protein in developing and postnatal heart leads to bradycardia, complete heart block and sudden death. Ultrastructural evidence indicates that the deficiency associated with the absence of calreticulin in the heart may be due to a defect in the development of the contractile apparatus and/or a defect in development of the conductive system as well as a metabolic abnormality. Collectively, we postulate that calreticulin and endoplasmic reticulum plays an important role in cardiac development and postnatal pathologies.
钙网蛋白是一种内质网驻留的Ca2+结合/储存伴侣蛋白。该蛋白在内质网中的钙网蛋白/钙连蛋白循环及质量控制途径中起关键作用。钙网蛋白缺乏会因心脏发育受损而致死。然而，在发育中和出生后的心脏中该蛋白过度表达会导致心动过缓、完全性心脏传导阻滞和猝死。超微结构证据表明，心脏中缺乏钙网蛋白相关的缺陷可能是由于收缩装置发育缺陷和/或传导系统发育缺陷以及代谢异常。总的来说，我们推测钙网蛋白和内质网在心脏发育及出生后病理过程中起重要作用。
