Humbert Sandrine, Saudou Frédéric
UMR 146 CNRS, Institut Curie, Bldg. 110, Centre Universitaire, 91405 Orsay Cedex, France.
Neuron. 2002 May 30;34(5):669-70. doi: 10.1016/s0896-6273(02)00715-8.
Transcriptional dysregulation appears as an emerging and unifying pathogenic mechanism in polyQ neurodegenerative disorders such as Spinocerebellar ataxias and Huntington's disease. It is unclear how cell death specificity occurs in these diseases. In this issue of Neuron, link polymerase II, a general component of the transcriptional machinery, to PQBP-1, a cerebellar enriched protein, thus providing insight into the selectivity of neuronal death in SCA1.
转录失调似乎是脊髓小脑共济失调和亨廷顿舞蹈症等多聚谷氨酰胺神经退行性疾病中一种新出现的统一致病机制。目前尚不清楚这些疾病中细胞死亡特异性是如何发生的。在本期《神经元》杂志中，将转录机制的一个通用成分——RNA聚合酶II，与一种小脑富集蛋白PQBP-1联系起来，从而为深入了解脊髓小脑共济失调1型（SCA1）中神经元死亡的选择性提供了思路。
