Immunological approaches to the characterization and diagnosis of mitochondrial disease
Immunological approaches to the characterization and diagnosis of mitochondrial disease
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DOI：
10.1016/j.mito.2004.07.006
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发表时间：
2004-09-01
期刊：
影响因子：
4.4
通讯作者：
Marusich, MF
中科院分区：
文献类型：
--
作者：
Capaldi, RA;Murray, J;Marusich, MF
单克隆抗体（mab）是线粒体疾病诊断和表征的重要工具。它们可用于免疫组织化学和/或Western blotting方法，以识别错误组装的OXPHOS复合物或丙酮酸脱氢酶缺乏症，其中大部分情况下未形成完整的复合物。基于抗体的方法的优点是它们可以定量，需要非常少量的组织样本，并且快速，简单且相对便宜。在这里，我们提供了目前可用的单克隆抗体的细节，并描述了使用患者成纤维细胞的免疫组织化学以及使用细胞培养或活检材料的Western blotting的优化方案。(C) 2004 Elsevier B.V.与线粒体研究学会。版权所有。
Monoclonal antibodies (mAbs) are important tools in the diagnosis and characterization of mitochondrial diseases. They can be used in immunohistochemical and/or Western blotting approaches to identify misassembled OXPHOS complexes or pyruvate dehydrogenase deficiencies where the intact complex is not formed which is the great majority of cases. The advantage of antibody based approaches is that they can be quantitative, require very small amounts of tissue sample and are fast, simple and relatively cheap to perform. Here we provide details of the mAbs currently available and describe optimized protocols for both immunohistochemistry using patient fibroblasts as well as Western blotting using either cell culture or biopsy material. (C) 2004 Elsevier B.V. and Mitochondria Research Society. All rights reserved.