A Figge David, Standaert David G
Center for Neurodegeneration and Experimental Therapeutics, Department of Neurology, The University of Alabama at Birmingham, Birmingham, AL, USA.
Center for Neurodegeneration and Experimental Therapeutics, Department of Neurology, The University of Alabama at Birmingham, Birmingham, AL, USA.
Neurobiol Dis. 2017 Jun;102:125-132. doi: 10.1016/j.nbd.2017.03.003. Epub 2017 Mar 9.
Levodopa (L-DOPA) remains the most effective pharmacological treatment for Parkinson Disease (PD) but its use is limited by the development of debilitating drug-related side effects, particularly L-DOPA induced dyskinesia (LID). LID is a consequence of long-term L-DOPA use, and in model systems is characterized by a "priming effect", whereby initial administrations of L-DOPA trigger a sensitized biochemical and transcriptional response upon subsequent dopaminergic stimulation. Preliminary studies into the mechanisms underlying this cellular memory have indicated an important role for epigenetic change but many of the downstream mechanisms remain unknown. The family of bromodomain and extraterminal (BET) proteins, which bind acetylated histones, play a critical effector role in the regulation of transcription. BET proteins have been implicated in several forms of neural plasticity, but their potential relevance to LID remains unexplored. Using the 6-OHDA rodent model of LID, we show that dyskinesia development induces alterations in BET protein expression along with enhanced occupation of sites at the promoter and enhancer regions of genes dysregulated during dyskinesia development. When BET function was blocked using the pharmacologic inhibitor JQ1, LID was prevented. In addition, we found that JQ1 treatment blocked the transcriptional upregulation of several immediate-early genes known to participate in the pathogenesis of dyskinesia. Together, these results demonstrate an essential role for BET protein activity as an epigenetic "reader" of the altered histone acetylation required for LID development and suggest that modulation of BET protein function is a potential therapeutic avenue for the prevention or reversal of LID in PD.
左旋多巴(L-DOPA)仍然是治疗帕金森病(PD)最有效的药物,但它的使用受到令人衰弱的药物相关副作用的限制,尤其是左旋多巴诱发的运动障碍(LID)。LID是长期使用L-DOPA的结果,在模型系统中其特征为“启动效应”,即L-DOPA的初始给药会在随后的多巴胺能刺激时引发敏感的生化和转录反应。对这种细胞记忆潜在机制的初步研究表明表观遗传变化起重要作用,但许多下游机制仍不清楚。与乙酰化组蛋白结合的溴结构域和额外末端(BET)蛋白家族在转录调控中起关键效应作用。BET蛋白与多种形式的神经可塑性有关,但其与LID的潜在相关性仍未得到探索。利用LID的6-OHDA啮齿动物模型,我们发现运动障碍的发展会诱导BET蛋白表达改变,同时在运动障碍发展过程中失调的基因的启动子和增强子区域的位点占据增加。当使用药理抑制剂JQ1阻断BET功能时,可预防LID。此外,我们发现JQ1处理可阻断几个已知参与运动障碍发病机制的即刻早期基因的转录上调。总之,这些结果证明BET蛋白活性作为LID发展所需的组蛋白乙酰化改变的表观遗传“读取器”起着至关重要的作用,并表明调节BET蛋白功能是预防或逆转PD中LID的潜在治疗途径。