Laboratory for Bone Metabolism, Key Laboratory for Space Bioscience and Biotechnology, School of Life Sciences, Northwestern Polytechnical University, Xi'an, Shaanxi 710072, PR China; Shenzhen Research Institution of Northwestern Polytechnical University, Shenzhen, 518057, PR China; Northwestern Polytechnical University-Hong Kong Baptist University Joint Research Centre for Translational Medicine on Musculoskeletal Health in Space, Xi'an, 710072, PR China.
Laboratory for Bone Metabolism, Key Laboratory for Space Bioscience and Biotechnology, School of Life Sciences, Northwestern Polytechnical University, Xi'an, Shaanxi 710072, PR China; Shenzhen Research Institution of Northwestern Polytechnical University, Shenzhen, 518057, PR China; Northwestern Polytechnical University-Hong Kong Baptist University Joint Research Centre for Translational Medicine on Musculoskeletal Health in Space, Xi'an, 710072, PR China.
Semin Cell Dev Biol. 2017 Sep;69:34-39. doi: 10.1016/j.semcdb.2017.06.005. Epub 2017 Jun 13.
Spectraplakins are multifunctional cytoskeletal linker proteins that act as important communicators, connecting cytoskeletal components with each other and to cellular junctions. Bullous pemphigoid antigen 1 (BPAG1)/dystonin is a member of spectraplakin family and expressed in various tissues. Alternative splicing of BPAG1 gene produces various isoforms with unique structure and domains. BPAG1 plays crucial roles in numerous biological processes, such as cytoskeleton organization, cell polarization, cell adhesion, and cell migration as well as signaling transduction. Genetic mutation of BPAG1 isoforms is the miscreant of epidermolysis bullosa and multifarious, destructive neurological diseases. In this review, we summarize the recent advances of BPAG1's role in various biological processes and in skin and neurological diseases.
光谱钙黏连蛋白是多功能细胞骨架连接蛋白，作为重要的通讯蛋白，将细胞骨架成分彼此连接，并与细胞连接连接。大疱性类天疱疮抗原 1（BPAG1）/ dystonin 是光谱钙黏连蛋白家族的成员，在各种组织中表达。BPAG1 基因的选择性剪接产生具有独特结构和结构域的各种异构体。BPAG1 在许多生物学过程中发挥着关键作用，如细胞骨架组织、细胞极化、细胞黏附和细胞迁移以及信号转导。BPAG1 异构体的遗传突变是表皮松解症和多种破坏性神经疾病的罪魁祸首。在这篇综述中，我们总结了 BPAG1 在各种生物学过程以及皮肤和神经疾病中的作用的最新进展。
