Investigating cell death mechanisms in amyotrophic lateral sclerosis using transcriptomics.

Investigating cell death mechanisms in amyotrophic lateral sclerosis using transcriptomics.
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DOI:
10.3389/fncel.2013.00259
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发表时间:
2013-12-17
影响因子:
5.3
通讯作者:
Shaw PJ
Shaw PJ
中科院分区:
医学2区
文献类型:
--
作者:
Heath PR;Kirby J;Shaw PJ

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肌萎缩性侧索硬化症(ALS)是一种运动神经元疾病,其特征在于来自运动皮层、脑干和脊髓的上运动神经元和下运动神经元的变性和丧失,尽管有证据表明在周围组织中存在其他细胞类型的进一步参与。通过使用微阵列技术的基因表达谱的转录组学分析使得能够确定退化组织中的细胞死亡模式。这项工作已经检查了基因表达水平的组织和个别细胞类型的散发性和家族性形式的疾病。此外,进一步的研究已经检查了中枢神经系统不同区域的神经元细胞的差异脆弱性。模型系统还提供了进一步的信息,以帮助解开导致疾病中运动神经元死亡的机制,并提供了新的见解。在这篇综述中,我们将描述这些研究中使用的方法,并描述它们如何有助于我们了解ALS的细胞死亡机制。
Amyotrophic lateral sclerosis (ALS) is a motor neuron disease characterized by degeneration and loss of upper and lower motor neurons from the motor cortex, brainstem and spinal cord although evidence is suggesting that there is further involvement of other cell types in the surrounding tissue. Transcriptomic analysis by gene expression profiling using microarray technology has enabled the determination of patterns of cell death in the degenerating tissues. This work has examined gene expression at the level of the tissue and individual cell types in both sporadic and familial forms of the disease. In addition, further studies have examined the differential vulnerability of neuronal cells in different regions of the central nervous system. Model systems have also provided further information to help unravel the mechanisms that lead to death of the motor neurons in disease and also provided novel insights. In this review we shall describe the methods that have been used in these investigations and describe how they have contributed to our knowledge of the cell death mechanisms in ALS.
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