Department of Immunology and Immunogenetics, Pathology Building, Southmead Hospital, Westbury-on-Trym, Bristol BS10 5NB, United Kingdom.
J Immunol Methods. 2014 Jan 15;402(1-2):71-5. doi: 10.1016/j.jim.2013.10.009. Epub 2013 Nov 12.
Complement dysregulation from an uncontrolled activation of the alternate pathway can be mediated by C3 Nephritic Factor and results in C3 glomerulopathy. Identification of C3 degradation products C3c and C3d in patient serum provides evidence of uncontrolled complement activation. It is possible to detect C3c and C3d in patient serum by an immunofixation assay which induces in vitro C3 degradation. The clinical performance of the immunofixation assay has been assessed by comparing the assay results with findings from immunostaining of kidney biopsies. The immunofixation assay is a simple and reliable technique for detection of C3 degradation on a widely available platform and can be used to provide corroborative evidence of acquired complement dysregulation in patients with C3 glomerulopathy.
补体失调可由旁路的不受控制的激活介导，可导致 C3 肾小球病。在患者血清中鉴定 C3 降解产物 C3c 和 C3d 提供了补体不受控制激活的证据。通过诱导体外 C3 降解的免疫固定测定法可以检测患者血清中的 C3c 和 C3d。通过将测定结果与肾脏活检免疫染色的结果进行比较，评估了免疫固定测定法的临床性能。免疫固定测定法是一种简单可靠的技术，可在广泛可用的平台上检测 C3 降解，并可用于为 C3 肾小球病患者获得性补体失调提供佐证证据。
