Immunobiology and Cancer Research Program, Oklahoma Medical Research Foundation, Oklahoma City, 73104, USA.
Acta Biochim Biophys Sin (Shanghai). 2012 Mar;44(3):187-96. doi: 10.1093/abbs/gmr105. Epub 2011 Nov 29.
Janus kinase (JAK)-signal transducer and activators of transcription (STAT) signaling pathways play crucial roles in lymphopoiesis. In particular, JAK3 has unique functions in the lymphoid system such that JAK3 ablation results in phenotypes resembling severe combined immunodeficiency syndrome. This review focuses on the biochemistry, immunological functions, and clinical significance of JAK3. Compared with other members of the JAK family, the biochemical properties of JAK3 are relatively less well characterized and thus largely inferred from studies of JAK2. Furthermore, new findings concerning the cross-talks between Notch and JAK signaling pathways through ubiquitin-mediated protein degradation are discussed in more detail.
Janus 激酶(JAK)-信号转导子和转录激活子(STAT)信号通路在淋巴发生中起着至关重要的作用。特别是,JAK3 在淋巴系统中具有独特的功能,因此 JAK3 缺失会导致类似于严重联合免疫缺陷综合征的表型。本综述重点介绍 JAK3 的生物化学、免疫学功能和临床意义。与 JAK 家族的其他成员相比,JAK3 的生化特性相对较差,因此主要是从 JAK2 的研究中推断出来的。此外,还更详细地讨论了 Notch 和 JAK 信号通路通过泛素介导的蛋白质降解相互作用的新发现。