Scott-Zaki P, Purkall D, Bigbee J, Ruddy S, Yu R K
Department of Biochemistry and Molecular Biophysics, Medical College of Virginia Campus, Virginia Commonwealth University, Richmond 23298-0614, USA.
J Neurol Sci. 1999 Jun 1;165(2):160-9. doi: 10.1016/s0022-510x(99)00104-5.
We used rat myelinated dorsal root ganglion (MDRG) cultures to study antibody and complement-mediated mechanisms of peripheral demyelinating diseases. Heat inactivated serum from a patient (LT) with peripheral neuropathy and a monoclonal IgM reactive with myelin-associated glycoprotein (anti-MAG) and sulfated glucuronosyl glycolipids (anti-SGGL) was used as an antibody source. Incubation of whole human serum (WHS) or WHS and anti-SGGL with MDRGs resulted in reduction of classical and alternative pathway hemolytic activities and the development of abnormal myelin sheaths. Incubation of MDRG cultures with C2-deficient serum showed activation of the alternative complement pathway. Classical pathway hemolytic activity was reduced when Factor B-depleted serum was incubated with MDRG cultures. The rat MDRG culture system provides a good model system of a peripheral nerve and has therefore been used by several investigators to study antibody and complement-mediated demyelination associated with peripheral neuropathies. However, our studies indicate a high degree of complement activation and membrane disruption of cultures incubated with WHS.
我们使用大鼠有髓背根神经节（MDRG）培养物来研究抗体和补体介导的周围性脱髓鞘疾病机制。来自一名患有周围神经病的患者（LT）的热灭活血清以及一种与髓鞘相关糖蛋白（抗MAG）和硫酸化葡糖醛酸糖脂（抗SGGL）反应的单克隆IgM被用作抗体来源。将全人血清（WHS）或WHS与抗SGGL与MDRG一起孵育，导致经典和替代途径溶血活性降低以及异常髓鞘的形成。用C2缺陷血清孵育MDRG培养物显示替代补体途径被激活。当用B因子耗尽的血清孵育MDRG培养物时，经典途径溶血活性降低。大鼠MDRG培养系统提供了一个良好的周围神经模型系统，因此被几位研究人员用于研究与周围神经病相关的抗体和补体介导的脱髓鞘。然而，我们的研究表明，用WHS孵育的培养物存在高度的补体激活和膜破坏。
