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Regulation of Adult Neurogenesis by the Fragile X Family of RNA Binding Proteins

机译:脆弱的X族RNA结合蛋白对成人神经发生的调控

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The fragile X mental retardation protein (FMRP) has an important role in neural development. Functional loss of FMRP in humans leads to fragile X syndrome, and it is the most common monogenetic contributor to intellectual disability and autism. FMRP is part of a larger family of RNA-binding proteins known as FXRs, which also includes fragile X related protein 1 (FXR1P) and fragile X related protein 2 (FXR2P). Despite the similarities of the family members, the functions of FXR1P and FXR2P in human diseases remain unclear. Although most studies focus on FMRP’s role in mature neurons, all three FXRs regulate adult neurogenesis. Extensive studies have demonstrated important roles of adult neurogenesis in neuroplasticity, learning, and cognition. Impaired adult neurogenesis is implicated in neuropsychiatric disorders, neurodegenerative diseases, and neurodevelopmental disorders. Interventions aimed at regulating adult neurogenesis are thus being evaluated as potential therapeutic strategies. Here, we review and discuss the functions of FXRs in adult neurogenesis and their known similarities and differences. Understanding the overlapping regulatory functions of FXRs in adult neurogenesis can give us insights into the adult brain and fragile X syndrome.
机译:脆弱的X智力低下蛋白(FMRP)在神经发育中具有重要作用。 FMRP在人体内的功能丧失会导致脆弱的X综合征,这是导致智障和自闭症的最常见的单基因病因。 FMRP是称为FXR的较大的RNA结合蛋白家族的一部分,其中还包括易碎X相关蛋白1(FXR1P)和易碎X相关蛋白2(FXR2P)。尽管家庭成员相似,但FXR1P和FXR2P在人类疾病中的功能仍不清楚。尽管大多数研究都集中于FMRP在成熟神经元中的作用,但所有这三种FXR均调节成人神经发生。广泛的研究表明成人神经发生在神经可塑性,学习和认知中的重要作用。成人神经发生受损与神经精神疾病,神经退行性疾病和神经发育疾病有关。因此,旨在调节成人神经发生的干预措施被评估为潜在的治疗策略。在这里,我们审查和讨论FXRs在成人神经发生中的功能及其已知的异同。了解FXR在成人神经发生中的重叠调节功能可以使我们深入了解成人大脑和脆性X综合征。

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