NEUROFILAMENT LIGHT AND POLYADENYLATED MESSENGER-RNA LEVELS ARE DECREASED IN AMYOTROPHIC-LATERAL-SCLEROSIS MOTOR-NEURONS
NEUROFILAMENT LIGHT AND POLYADENYLATED MESSENGER-RNA LEVELS ARE DECREASED IN AMYOTROPHIC-LATERAL-SCLEROSIS MOTOR-NEURONS
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DOI：
10.1097/00005072-199405000-00002
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发表时间：
1994-05-01
影响因子：
3.2
通讯作者：
PERCY, ME
中科院分区：
文献类型：
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作者：
BERGERON, C;BERICMASKAREL, K;PERCY, ME
肌萎缩侧索硬化症 (ALS) 中运动神经元的核周和近端轴突中存在大量神经丝状积累，表明这种丰富的细胞骨架蛋白的表达可能会改变。我们对 6 例散发性 ALS 病例和 6 例对照患者的低分子量神经丝亚基 (NF-L) 信使 RNA 进行了定量原位杂交。我们发现 ALS 患者前角细胞中的 NF-L mRNA 水平下降了 41% (p < 0.02)，α 运动神经元中的 NF-L mRNA 水平下降了 60% (p 小于或等于 0.01)。这种改变可能代表对轴突或神经元损伤的非特异性反应，或者反映残余正常运动神经元的再生活动。在带有球状体的运动神经元中，NF-L mRNA 水平始终较低（在第三和第四四分位数），表明在 ALS 中观察到的神经丝积累不太可能是 NF-L 基因过度表达的结果。 ALS 中前角细胞中总神经元聚腺苷酸化 mRNA 水平也降低了 50%（p = 0.02），α 运动神经元中则降低了 48%（p 小于或等于 0.05），这可能反映了患病运动神经元中选定 mRNA 种类的减少。
The presence of large neurofilamentous accumulations in the perikaryon and proximal axon of motor neurons in amyotrophic lateral sclerosis (ALS) suggests that the expression of this abundant cytoskeletal protein may be altered. We performed quantitative in situ hybridization for the low molecular weight neurofilament subunit (NF-L) messenger RNA in six cases of sporadic ALS and six controls. We found a 41% decrease (p < 0.02) in the NF-L mRNA levels in anterior horn cells in ALS, with a 60% decrease (p less than or equal to 0.01) in alpha motor neurons. This alteration may represent a non-specific response to axonal or neuronal injury or, alternatively, reflect the regenerative activity of residual normal motor neurons. NF-L mRNA levels were consistently low (in the third and fourth quartiles) in spheroid-bearing motor neurons, indicating that the neurofilamentous accumulations observed in ALS are not likely the result of overexpression of the NF-L gene. Total neuronal polyadenylated mRNA levels were also 50% lower (p = 0.02) in anterior horn cells and 48% lower (p less than or equal to 0.05) in alpha motor neurons in ALS, possibly reflecting a decrease in selected mRNA species in diseased motor neurons.