Signal Recognition Particle in Human Diseases.
Signal Recognition Particle in Human Diseases.
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DOI：
10.3389/fgene.2022.898083
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发表时间：
2022
影响因子：
3.7
通讯作者：
中科院分区：
文献类型：
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作者：
信号识别颗粒（SRP）是一种具有双重功能的核糖核蛋白复合物。它通过共翻译将具有信号序列的蛋白质靶向内质网 (ER)，并保护其 mRNA 免遭降解。如果 SRP 耗尽或无法识别信号序列，则会激活异常蛋白产生调节 (RAPP)，从而导致分泌蛋白 mRNA 丢失。如果 SRP 识别底物但无法将其靶向 ER，则它们可能会错误定位或降解。所有这些事件都会对蛋白质生物发生产生巨大影响，激活蛋白质质量控制途径，并对细胞生理学产生压力，并可能导致疾病的发病机制。事实上，SRP 功能障碍与许多不同的人类疾病有关，包括： 先天性中性粒细胞减少症；特发性炎症性肌病;病毒、原虫和朊病毒感染；和癌症。在这项工作中，我们分析了SRP失败引起的疾病并讨论了其可能的分子机制。
The signal recognition particle (SRP) is a ribonucleoprotein complex with dual functions. It co-translationally targets proteins with a signal sequence to the endoplasmic reticulum (ER) and protects their mRNA from degradation. If SRP is depleted or cannot recognize the signal sequence, then the Regulation of Aberrant Protein Production (RAPP) is activated, which results in the loss of secretory protein mRNA. If SRP recognizes the substrates but is unable to target them to ER, they may mislocalize or degrade. All these events lead to dramatic consequence for protein biogenesis, activating protein quality control pathways, and creating pressure on cell physiology, and might lead to the pathogenesis of disease. Indeed, SRP dysfunction is involved in many different human diseases, including: congenital neutropenia; idiopathic inflammatory myopathy; viral, protozoal, and prion infections; and cancer. In this work, we analyze diseases caused by SRP failure and discuss their possible molecular mechanisms.
影响因子：
4.6
作者：
Zhang Q;Ma J;Liu H;He D;Chen L;Wu H;Jiang H;Lu Q;Bai S
通讯作者：
Bai S