良好的细胞内环境稳态是运动神经元存活的关键吗？

Is good housekeeping the key to motor neuron survival?

作者信息

Talbot Kevin, Davies Kay E

机构信息

MRC Functional Genetics Unit, Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX1 3QX, UK.

出版信息

Cell. 2008 May 16;133(4):572-4. doi: 10.1016/j.cell.2008.05.002.

DOI:10.1016/j.cell.2008.05.002

PMID:18485864

Abstract

Spinal muscular atrophy (SMA) is caused by a drastic reduction in the ubiquitously expressed SMN protein, which is critical for the correct assembly of the snRNP complexes required for RNA splicing. However, it is unclear why loss of SMN and altered snRNP assembly only seem to affect motor neurons. Reporting in this issue, Zhang et al. (2008) challenge prior assumptions about the housekeeping function of SMN and demonstrate that loss of SMN leads to highly tissue-specific effects on splicing.

摘要

脊髓性肌萎缩症（SMA）是由普遍表达的生存运动神经元（SMN）蛋白急剧减少引起的，该蛋白对于RNA剪接所需的小核核糖核蛋白（snRNP）复合体的正确组装至关重要。然而，目前尚不清楚为何SMN的缺失和snRNP组装的改变似乎仅影响运动神经元。在本期发表的报告中，Zhang等人（2008年）对之前关于SMN管家功能的假设提出了质疑，并证明SMN的缺失会对剪接产生高度组织特异性的影响。