Clinical Neurobiology, Institute of Biomedical and Clinical Science, Peninsula Medical School, St. Luke's Campus, Heavitree Road, Exeter EX1 2LU, UK.
J Mol Biol. 2010 Sep 3;401(5):681-9. doi: 10.1016/j.jmb.2010.06.058. Epub 2010 Jul 8.
Childhood spinal muscular atrophy is caused by a reduced expression of the survival motor neuron (SMN) protein. SMN has been implicated in the axonal transport of beta-actin mRNA in both primary and transformed neuronal cell lines, and loss of this function could account, at least in part, for spinal muscular atrophy onset and pathological specificity. Here we have utilised a targeted screen to identify mRNA associated with SMN, Gemin2 and Gemin3 in the cytoplasm of a human neuroblastoma cell line, SHSY5Y. Importantly, we have provided the first direct evidence that beta-actin mRNA is present in SMN cytoplasmic complexes in SHSY5Y cells.
儿童脊髓性肌萎缩症是由生存运动神经元(SMN)蛋白表达减少引起的。SMN 已被牵连到原代和转化神经元细胞系中β-肌动蛋白 mRNA 的轴突运输中,而这种功能的丧失至少可以部分解释脊髓性肌萎缩症的发病和病理特异性。在这里,我们利用靶向筛选来鉴定与 SMN、Gemin2 和 Gemin3 相关的 mRNA 在人神经母细胞瘤细胞系 SHSY5Y 的细胞质中。重要的是,我们提供了第一个直接证据表明β-肌动蛋白 mRNA存在于 SHSY5Y 细胞中的 SMN 细胞质复合物中。