Basile Umberto, Gulli Francesca, Gragnani Laura, Napodano Cecilia, Pocino Krizia, Rapaccini Gian Ludovico, Mussap Michele, Zignego Anna Linda
Department of Laboratory Medicine of the Catholic University of Sacred Heart, Rome, Italy.
Department of Laboratory Medicine of the Catholic University of Sacred Heart, Rome, Italy.
J Immunol Methods. 2017 Dec;451:11-19. doi: 10.1016/j.jim.2017.09.005. Epub 2017 Sep 18.
The production of antibodies is accompanied by a slight excess of synthesis of κ and λ immunoglobulin light chains; small amounts of them are released in the peripheral blood and can also be found in various body fluids, such as synovial fluid, cerebrospinal fluid, urine and saliva. They are rapidly filtered by the glomerulus and >99% are reabsorbed from the cells of the proximal convoluted tubule, making them present in the urine in only trace amounts. The production of an excess of protein without a reason or a specific function in a biological system is rare. Free light chains, considered for years a waste product of Ig synthesis, are currently known to be very active molecules, able to bind antigens as well as whole immunoglobulin and helping to develop specific antibody affinity. The ability of free light chains to activate mast cells and then become an active part of the pathogenic mechanisms of chronic inflammatory diseases has increased interest in their clinical use, both as an attractive therapeutic target or as a biochemical marker of disease evolution or remission. This is an overview of relevant scientific interest that immunoglobulin light chains κ and λ have attracted over the years, a report on the progress in knowledge about their structure and function, with a special focus on their biological meaning and potential clinical utility in different diseases.
抗体的产生伴随着κ和λ免疫球蛋白轻链合成的轻微过量；它们中的少量会释放到外周血中，也能在各种体液中被发现，如滑液、脑脊液、尿液和唾液。它们会迅速被肾小球滤过，超过99%会从近端曲管的细胞中被重吸收，使得它们仅以痕量存在于尿液中。在生物系统中无端产生过量蛋白质或无特定功能的情况很少见。游离轻链多年来一直被认为是免疫球蛋白合成的废物，目前已知它们是非常活跃的分子，能够结合抗原以及完整的免疫球蛋白，并有助于形成特异性抗体亲和力。游离轻链激活肥大细胞并进而成为慢性炎症性疾病致病机制的活跃部分的能力，增加了人们对其临床应用的兴趣，既将其作为一个有吸引力的治疗靶点，也作为疾病进展或缓解的生化标志物。这是对免疫球蛋白轻链κ和λ多年来所吸引的相关科学兴趣的概述，是关于它们结构和功能知识进展的报告，特别关注它们在不同疾病中的生物学意义和潜在临床效用。
