摘要
伴肌动蛋白相关锚定蛋白(nebulin-related-anchoring protein,NRAP)是一种多结构域骨架蛋白,特异性表达在心肌闰盘和骨骼肌肌腱的连接处。在肌原纤维的组装过程中起到重要作用,并参与将肌原纤维的末端肌动蛋白丝锚定在细胞膜上,使张力从肌原纤维传递到细胞外基质。迄今为止有关NRAP临床研究报道多见于肌肉病变,但近年来有新的研究发现NRAP基因突变与扩张型心肌病有关。本文就NRAP的结构、生物学功能以及相关疾病的研究进展作一阐述。
Nebulin-related-anchoring protein(NRAP)is a multi-domain skeletal protein that is specifically expressed at the myocardial intercalated disc and the tendon junction of skeletal muscle.It plays an important role in the assembly process of myofibrils and participates in anchoring the terminal actin filaments of myofibrils to the cell membrane to transfer tension from myofibrils to extracellular matrix.Up to now,clinical research reports on NRAP are mostly found in muscular lesions,but in recent years,there are new reports of NRAP gene mutation and dilated cardiomyopathy.This article reviews the structure,biological functions and research progress of NRAP.
作者
周明
杨世伟
ZHOU Ming;YANG Shi-wei(Department of Cardiology,Children’s Hospital of Nanjing Medical University,Nanjing 210019,Jiangsu,China)
出处
《心脏杂志》
CAS
2020年第3期315-318,共4页
Chinese Heart Journal
基金
国家自然科学基金项目资助(81670284)
南京市医学科技发展资金青年人才工程项目资助(QRX17024)
南京市科委国际合作课题项目资助(201715057)。
