Bleesing Jack
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA; Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
J Immunol Methods. 2025 Feb;537:113812. doi: 10.1016/j.jim.2025.113812. Epub 2025 Jan 26.
Abnormal lymphocyte homeostasis underly several Inborn Errors of Immunity (IEoI). In vitro assessment of lymphocyte homeostasis is achieved by specific apoptosis assays reflective of specific homeostasis programs and pathways that are mediated through specific proteins. This review discusses those programs, pathways and proteins and describes the development and use of the in vitro Fas-mediated apoptosis assay, as it relates to the IEoI Autoimmune Lymphoproliferative Syndrome (ALPS) and describes other disorders of lymphocyte homeostasis in the context of other forms of in vitro apoptosis assessment.
异常的淋巴细胞稳态是多种先天性免疫缺陷（IEoI）的基础。通过反映特定稳态程序和由特定蛋白质介导的途径的特异性凋亡检测，可在体外评估淋巴细胞稳态。本综述讨论了这些程序、途径和蛋白质，并描述了体外Fas介导的凋亡检测的开发和应用，因为它与IEoI自身免疫性淋巴细胞增生综合征（ALPS）相关，并在其他形式的体外凋亡评估背景下描述了淋巴细胞稳态的其他紊乱情况。
