Department of Public Health/Geriatrics, Rudbeck Laboratory, Uppsala University, Uppsala, Sweden.
BioArctic Neuroscience AB, Stockholm, Sweden.
Neurobiol Dis. 2014 Sep;69:134-43. doi: 10.1016/j.nbd.2014.05.009. Epub 2014 May 20.
Several lines of evidence suggest that accumulation of aggregated alpha-synuclein (α-synuclein) in the central nervous system (CNS) is an early pathogenic event in Parkinson's disease and other Lewy body disorders. In recent years, animal studies have indicated immunotherapy with antibodies directed against α-synuclein as a promising novel treatment strategy. Since large α-synuclein oligomers, or protofibrils, have been demonstrated to possess pronounced cytotoxic properties, such species should be particularly attractive as therapeutic targets. In support of this, (Thy-1)-h[A30P] α-synuclein transgenic mice with motor dysfunction symptoms were found to display increased levels of α-synuclein protofibrils in the CNS. An α-synuclein protofibril-selective monoclonal antibody (mAb47) was evaluated in this α-synuclein transgenic mouse model. As measured by ELISA, 14month old mice treated for 14weeks with weekly intraperitoneal injections of mAb47 displayed significantly lower levels of both soluble and membrane-associated protofibrils in the spinal cord. Besides the lower levels of pathogenic α-synuclein demonstrated, a reduction of motor dysfunction in transgenic mice upon peripheral administration of mAb47 was indicated. Thus, immunotherapy with antibodies targeting toxic α-synuclein species holds promise as a future disease-modifying treatment in Parkinson's disease and related disorders.
有几条证据表明,α-突触核蛋白(α-synuclein)在中枢神经系统(CNS)中的聚集是帕金森病和其他路易体疾病的早期致病事件。近年来,动物研究表明,针对α-synuclein 的免疫疗法是一种很有前途的新型治疗策略。由于已经证明大型α-synuclein 寡聚体或原纤维具有明显的细胞毒性,因此这些物质应该特别有吸引力作为治疗靶点。支持这一点的是,具有运动功能障碍症状的(Thy-1)-h[A30P]α-synuclein 转基因小鼠在中枢神经系统中显示出α-synuclein 原纤维的水平升高。在这种α-synuclein 转基因小鼠模型中评估了一种α-synuclein 原纤维选择性单克隆抗体(mAb47)。通过 ELISA 测量,用每周一次的腹腔内注射 mAb47 治疗 14 周的 14 个月大的小鼠,脊髓中可溶性和膜相关原纤维的水平显著降低。除了证明致病性α-synuclein 的水平降低外,还表明在转基因小鼠中通过外周给予 mAb47 可以减少运动功能障碍。因此,针对毒性α-synuclein 物质的免疫疗法作为帕金森病和相关疾病的未来疾病修饰治疗具有很大的希望。