Role of stress granules and RNA-binding proteins in neurodegeneration: a mini-review.
Role of stress granules and RNA-binding proteins in neurodegeneration: a mini-review.
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作者：
Vanderweyde T;Youmans K;Liu-Yesucevitz L;Wolozin B
真核生物的应激反应涉及非管家蛋白的翻译抑制和不必要的mRNA转录物到应激颗粒（SG）的隔离。该过程依赖于mRNA结合蛋白（RBP），其通过RNA识别基序与加帽mRNA转录物相互作用，并通过疏水聚甘氨酸结构域表现出可逆聚集，其中一些与酵母朊病毒蛋白同源。RBP的活性和聚集在未折叠蛋白质疾病的背景下似乎是重要的。这些RBP突变可导致家族性运动神经元疾病和家族性痴呆的发现表明这些基因对神经元变性的重要性。与RBP突变相关的一些疾病包括：肌萎缩性侧索硬化症（ALS）、额颞叶痴呆和脊髓性肌萎缩症（SMA）。这些RBP还与其他神经退行性疾病中的病理结构相关，包括亨廷顿舞蹈病、克-雅二氏病和阿尔茨海默病。有趣的是，RBPs的蛋白质水平在整个衰老过程中都会发生变化，并且可能与其他年龄相关的疾病有关，例如2型糖尿病。SG途径和与神经退行性疾病相关的蛋白质之间的联系表明了两个过程中共同途径的潜在作用，例如参与翻译控制的途径，并突出了神经退行性疾病治疗干预的潜在新靶点。
The eukaryotic stress response involves translational suppression of non-housekeeping proteins and the sequestration of unnecessary mRNA transcripts into stress granules (SGs). This process is dependent on mRNA binding proteins (RBPs) that interact with capped mRNA transcripts through RNA recognition motifs, and exhibit reversible aggregation through hydrophobic poly-glycine domains, some of which are homologous to yeast prion proteins. The activity and aggregation of RBPs appears to be important in the context of unfolded protein diseases. The discovery that mutations in these RBPs can cause familial motor neuron diseases and familial dementias indicates the importance of these genes to neuronal degeneration. Some disorders linked to mutations in RBPs include: amyotrophic lateral sclerosis (ALS), frontotemporal dementia and spinal muscular atrophy (SMA). These RBPs also associate with pathological structures in other neurodegenerative diseases, including Huntington’s chorea, Creutzfeld-Jacob disease, and Alzheimer’s disease. Interestingly, protein levels of RBPs change across the aging spectrum and may be linked to other age-related disorders, such as type 2 diabetes. The link between SG pathways and proteins linked to neurodegenerative diseases suggests a potential role for common pathways in both processes, such as those involved in translational control, and highlights potentially novel targets for therapeutic intervention in neurodegenerative diseases.
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影响因子：
64.8
作者：
Moreno, Julie A.;Radford, Helois;Peretti, Diego;Steinert, Joern R.;Verity, Nicholas;Martin, Maria Guerra;Halliday, Mark;Morgan, Jason;Dinsdale, David;Ortori, Catherine A.;Barrett, David A.;Tsaytler, Pavel;Bertolotti, Anne;Willis, Anne E.;Bushell, Martin;Mallucci, Giovanna R.
通讯作者：
Mallucci, Giovanna R.
影响因子：
3.7
作者：
Liu-Yesucevitz L;Bilgutay A;Zhang YJ;Vanderweyde T;Citro A;Mehta T;Zaarur N;McKee A;Bowser R;Sherman M;Petrucelli L;Wolozin B
通讯作者：
Wolozin B
影响因子：
16.2
作者：
Hoover, Brian R.;Reed, Miranda N.;Su, Jianjun;Penrod, Rachel D.;Kotilinek, Linda A.;Grant, Marianne K.;Pitstick, Rose;Carlson, George A.;Lanier, Lorene M.;Yuan, Li-Lian;Ashe, Karen H.;Liao, Dezhi
通讯作者：
Liao, Dezhi
DOI：
10.18632/aging.100073
发表时间：
2009-07-26
期刊：
Aging
影响因子：
--
作者：
Masuda K;Marasa B;Martindale JL;Halushka MK;Gorospe M
通讯作者：
Gorospe M
RNA结合蛋白在癌症中的多功能性。
影响因子：
--
作者：
Wurth L
通讯作者：
Wurth L