细胞毒性T细胞
生物
免疫学
肌动蛋白细胞骨架
免疫系统
细胞生物学
细胞骨架
细胞
遗传学
体外
作者
Javier Chinen,Yousef R. Badran,Raif S. Geha,Janet Chou,Ari J. Fried
标识
DOI:10.1016/j.jaci.2017.07.023
摘要
Advances in basic immunology in 2016 included studies that further characterized the role of different proteins in the differentiation of effector T and B cells, including cytokines and proteins involved in the actin cytoskeleton. Regulation of granule formation and secretion in cytotoxic cells was also further described by examining patients with familial hemophagocytic lymphohistiocytosis. The role of prenylation in patients with mevalonate kinase deficiency leading to inflammation has been established. We reviewed advances in clinical immunology, as well as new approaches of whole-genome sequencing and genes newly reported to be associated with immunodeficiency, such as linker of activation of T cells (LAT) ; B-cell CLL/lymphoma 11B (BCL11B) ; RGD, leucine-rich repeat, tropomodulin domain, and proline-rich domain–containing protein (RLTPR) ; moesin; and Janus kinase 1 (JAK1) . Trials of hematopoietic stem cell transplantation and gene therapy for primary immunodeficiency have had relative success; the use of autologous virus-specific cytotoxic T cells has proved effective as well. New medications are being explored, such as pioglitazone, which is under study for its role in enhancing the oxidative burst in patients with chronic granulomatous disease. Development of vaccines for HIV infection continues to provide insight into the immune response against a virus with an extraordinary mutation rate.
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