Department of Neurology, Emory University, Atlanta, Georgia 30322, USA.
J Proteome Res. 2009 Nov;8(11):5069-79. doi: 10.1021/pr900474t.
Neurodegenerative diseases are often defined pathologically by the presence of protein aggregates. These aggregates, including amyloid plaques in Alzheimer's disease (AD), result from the abnormal accumulation and processing of proteins, and may ultimately lead to neuronal dysfunction and cell death. To date, conventional biochemical studies have revealed abundant core components in protein aggregates. However, rapidly improving proteomics technologies offer opportunities to revisit pathologic aggregate composition, and to identify less abundant but potentially important functional molecules that participate in neurodegeneration. The purpose of this study was to establish a proteomic strategy for the profiling of neurodegenerative disease tissues for disease-specific changes in protein abundance. Using high resolution liquid chromatography coupled with tandem mass spectrometry (LC-MS/MS), we analyzed detergent-insoluble frontal cortex samples from AD and unaffected control cases. In addition, we analyzed samples from frontotemporal lobar degeneration (FTLD) cases to identify AD-specific changes not present in other neurodegenerative diseases. We used a labeling-free quantification technique to compare the abundance of identified peptides in the samples based on extracted ion current (XIC) of their corresponding ions. Of the 512 identified proteins, quantitation demonstrated significant changes in 81 AD-specific proteins. Following additional manual filtering, 11 proteins were accepted with high confidence as increased in AD compared to control and FTLD brains, including beta-amyloid, tau and apolipoprotein E, all well-established AD-linked proteins. In addition, we identified and validated the presence of serine protease 15, ankyrin B, and 14-3-3 eta in the detergent-insoluble fraction. Our results provide further evidence for the capacity of proteomics applications to identify conserved sets of disease-specific proteins in AD, to enhance our understanding of disease pathogenesis, and to deliver new candidates for the development of effective therapies for this, and other, devastating neurodegenerative disorders.
神经退行性疾病通常通过蛋白质聚集体的存在在病理学上进行定义。这些聚集体,包括阿尔茨海默病(AD)中的淀粉样斑块,是由蛋白质的异常积累和处理引起的,最终可能导致神经元功能障碍和细胞死亡。迄今为止,传统的生化研究已经揭示了蛋白质聚集体中的丰富核心成分。然而,快速发展的蛋白质组学技术为重新审视病理性聚集体的组成以及鉴定参与神经退行性变的潜在重要功能分子提供了机会,这些分子的丰度较低。本研究的目的是建立一种蛋白质组学策略,用于分析神经退行性疾病组织中的蛋白质丰度变化,以确定疾病特异性变化。我们使用高分辨率液相色谱-串联质谱联用(LC-MS/MS)分析了 AD 和无影响对照病例的额皮质不溶性去污剂样本。此外,我们还分析了额颞叶变性(FTLD)病例的样本,以鉴定在其他神经退行性疾病中不存在的 AD 特异性变化。我们使用无标记定量技术,根据相应离子的提取离子电流(XIC)比较样本中鉴定肽的丰度。在鉴定的 512 种蛋白质中,定量分析显示 81 种 AD 特异性蛋白质的丰度发生了显著变化。经过进一步的手动过滤,有 11 种蛋白质被认为是 AD 脑与对照和 FTLD 脑相比增加的蛋白,具有较高的置信度,包括β-淀粉样蛋白、tau 和载脂蛋白 E,这些都是公认的与 AD 相关的蛋白。此外,我们还鉴定并验证了丝氨酸蛋白酶 15、锚蛋白 B 和 14-3-3eta 在不溶性去污剂部分的存在。我们的研究结果进一步证明了蛋白质组学应用在 AD 中鉴定保守的疾病特异性蛋白质集的能力,以增强我们对疾病发病机制的理解,并为这种疾病和其他破坏性神经退行性疾病的有效治疗方法的开发提供新的候选物。