Tsujimoto Y
Osaka University Medical School, Biomedical Research Center, Department of Medical Genetics, CREST of Japan Science and Technology Corp., Suita.
J Neural Transm Suppl. 2000(58):41-52. doi: 10.1007/978-3-7091-6284-2_4.
Apoptosis is an essential physiological cell death for selective elimination of cells, involved in a variety of biological events including morphogenesis, cell turn over and removal of harmful cells. Disruption of the regulation of apoptosis may result in various diseases, including cancer and autoimmune diseases both associated with inhibition of apoptosis and various degenerative disorders associated with enhancement of apoptosis, and therefore, apoptosis is an important theme in the medical field. Apoptosis is driven by a family of cysteine proteases, called caspases and regulated by a Bcl-2 family of proteins, which is the best characterized apoptosis regulators. The Bcl-2 family consists of anti-apoptotic and pro-apoptotic members, and some members are implicated in cancer and nuronal diseases. Here, I overview the mechanism of how Bcl-2 family proteins regulate cell death, and how they are implicated in human diseases, particularly focusing on role of Bcl-2 in spinal muscular atropy.
细胞凋亡是一种重要的生理性细胞死亡,用于选择性清除细胞,参与包括形态发生、细胞更新和清除有害细胞在内的多种生物学事件。细胞凋亡调控的破坏可能导致各种疾病,包括与细胞凋亡抑制相关的癌症和自身免疫性疾病,以及与细胞凋亡增强相关的各种退行性疾病,因此,细胞凋亡是医学领域的一个重要主题。细胞凋亡由一类称为半胱天冬酶的半胱氨酸蛋白酶驱动,并受Bcl-2蛋白家族调节,该家族是特征最明确的细胞凋亡调节因子。Bcl-2家族由抗凋亡和促凋亡成员组成,一些成员与癌症和神经疾病有关。在此,我概述Bcl-2家族蛋白如何调节细胞死亡的机制,以及它们如何与人类疾病相关,特别关注Bcl-2在脊髓性肌萎缩症中的作用。