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| Filamin B在骨骼系统中的研究进展 |
| Research progress of Filamin B in skeletal system |
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| DOI：10.3969/j.issn.1006.7108.2023.04.026 |
| 中文关键词: 细丝蛋白B （FLNB） 调控 骨骼系统 |
| 英文关键词:filament protein B (FLNB) regulate skeletal system |
| 基金项目:国家自然科学基金项目( 81860746，82060411)；新疆维吾尔自治区自然科学基金重点项目（2021D01D21，2021D01D19） |
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| 中文摘要: |
| 细丝蛋白B（Filamin B，FLNB）是一种大的二聚体肌动蛋白结合蛋白，是丝蛋白（Filamins）家族中的一员。其可将肌动蛋白细胞骨架细丝交联成动态结构，为细胞运动和信号传递提供了重要的支架。然当FLNB发生突变后，可导致严重的遗传性骨骼相关疾病。基于FLNB在骨骼系统中突变所致疾病的基础和临床研究，本文通过从FLNB基因的生物功能及其突变后影响骨骼系统发育和所致疾病的致病机制入手，对FLNB在骨骼系统中的研究进行综述，提供FLNB在骨骼系统中最新的研究进展。 |
| 英文摘要: |
| Filamin B (FLNB) is a large dimeric actin-binding protein that belongs to the filament family of proteins. It crosslinks actin cytoskeletal filaments into dynamic structures, providing an important scaffold for cell motility and signaling. However, when FLNB is mutated, it can lead to severe hereditary skeletal-related diseases. Based on the basic and clinical studies of diseases caused by FLNB mutations in the skeletal system, this study provides a review of FLNB research in the skeletal system by looking at the biological functions of FLNB genes and their pathogenic mechanisms that affect the development of the skeletal system and cause diseases after mutation, and provides the latest research progress of FLNB in the skeletal system. |
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