Li Linyang, Gu Huining, Qiu Xiaoyan, Huang Jing
Department of Immunology, School of Basic Medical Sciences, and NHC Key Laboratory of Medical Immunology, Peking University, Beijing 100191, China.
PUHSC Primary Immunodeficiency Research Center, School of Basic Medical Sciences, Peking University, Beijing 100191, China.
Int J Mol Sci. 2025 Aug 6;26(15):7607. doi: 10.3390/ijms26157607.
Immunoglobulin light chains are essential components of intact immunoglobulins, traditionally believed to be produced exclusively by B cells. Physiologically, excess light chains not assembled into intact antibodies exist as free light chains (FLCs). Increasingly recognized as important biomarkers for diseases such as multiple myeloma, systemic amyloidosis, and light chain-related renal injuries, FLCs have also been shown in recent decades to originate from non-B cell sources, including epithelial and carcinoma cells. This review primarily focuses on novel non-B cell-derived FLCs, which challenge the conventional paradigms. It systematically compares B cell-derived and non-B cell-derived FLCs, analyzing differences in genetic features, physicochemical properties, and functional roles in both health and disease. By elucidating the distinctions and similarities in their nature as immune regulators and disease mediators, we highlight the significant clinical potential of FLCs, particularly non-B cell-derived FLCs, for novel diagnostic and therapeutic strategies.
免疫球蛋白轻链是完整免疫球蛋白的重要组成部分，传统上认为其仅由B细胞产生。在生理状态下，未组装成完整抗体的过量轻链以游离轻链（FLC）的形式存在。近年来，FLC越来越被认为是多发性骨髓瘤、系统性淀粉样变性和轻链相关肾损伤等疾病的重要生物标志物，近几十年来还发现其来源于非B细胞来源，包括上皮细胞和癌细胞。本综述主要关注新型非B细胞来源的FLC，它们挑战了传统范式。系统比较了B细胞来源和非B细胞来源的FLC，分析了它们在遗传特征、理化性质以及在健康和疾病中的功能作用方面的差异。通过阐明它们作为免疫调节剂和疾病介质的本质区别和相似之处，我们强调了FLC，特别是非B细胞来源的FLC在新型诊断和治疗策略方面的巨大临床潜力。
Zotero 插件