近日来，一项研究表明，肿瘤细胞中相同的细胞凋亡抑制蛋白也可以表达在引起自身免疫疾病的细胞中。乔治华盛顿大学医学与科学院神经科主席医学博士Henry Kaminski ，与来自罗斯威尔帕克癌症研究所的同事们在Kusner实验室针对这个项目一起进行合作研究。他们发现了生存素，这是一种细胞凋亡的抑制蛋白，生存素在患有重症肌无力的病人体内淋巴细胞的白细胞中表达，但不在正常的个体中表达。这种情况也发生在动物模型的案例中。重症肌无力是一种严重的肌肉疾病，这种疾病会导致病人非常虚弱，以至于病人必须使用呼吸机才能生存。

“患有重症肌无力的人类也在自身反应性淋巴细胞中表达生存素，”Kusner说，“我们发现这些自身反应性淋巴细胞有自身免疫疾病功能障碍的特点。”

研究组使用疫苗技术能消除生存素表达类细胞，并且证明使用疫苗技术能改善患有重症肌无力的动物模型。Kusner的实验团队将为用生存素抑制子作为一种治疗药物来靶向治疗病人而努力奋斗。

“这项研究打开了一种治疗重症肌无力的新方法。和其他自体免疫疾病一样，传统疗法可能会改善该疾病状况，但会伴有很多病发症的发生。这个发现会为成千上万患自体免疫疾病的美国人提供另一种可行的治疗方法。”Kaminski说。

原文链接：http：//medicalxpress.com/news/2014-07-pathogenic-autoimmune-disorders-cancer.html#userconsent#

Survivin as a Potential Mediator to Support Autoreactive Cell Survival in Myasthenia Gravis: A Human and Animal Model Studyt

Linda L. Kusner mail,Michael J. Ciesielski,Alexander Marx,Henry J. Kaminski,Robert A. Fenstermaker

The mechanisms that underlie the development and maintenance of autoimmunity in myasthenia gravis are poorly understood. In this investigation, we evaluate the role of survivin, a member of the inhibitor of apoptosis protein family, in humans and in two animal models. We identified survivin expression in cells with B lymphocyte and plasma cells markers, and in the thymuses of patients with myasthenia gravis. A portion of survivin-expressing cells specifically bound a peptide derived from the alpha subunit of acetylcholine receptor indicating that they recognize the peptide. Thymuses of patients with myasthenia gravis had large numbers of survivin-positive cells with fewer cells in the thymuses of corticosteroid-treated patients. Application of a survivin vaccination strategy in mouse and rat models of myasthenia gravis demonstrated improved motor assessment, a reduction in acetylcholine receptor specific autoantibodies, and a retention of acetylcholine receptor at the neuromuscular junction, associated with marked reduction of survivin-expressing circulating CD20+ cells. These data strongly suggest that survivin expression in cells with lymphocyte and plasma cell markers occurs in patients with myasthenia gravis and in two animal models of myasthenia gravis. Survivin expression may be part of a mechanism that inhibits the apoptosis of autoreactive B cells in myasthenia gravis and other autoimmune disorders.