Developmental Dynamics of Rett Syndrome.
Developmental Dynamics of Rett Syndrome.
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DOI：
10.1155/2016/6154080
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发表时间：
2016
影响因子：
3.1
通讯作者：
Sur M
中科院分区：
文献类型：
--
作者：
Feldman D;Banerjee A;Sur M
Rett综合征长期以来被认为是一种出生后发育的疾病，其表型仅在发育后期和成年期表现出来。最近的各种证据表明，雷特综合征的表型存在于大脑发育的最早阶段，包括定义神经发生、迁移和模式化的发育阶段以及突触和回路发育和可塑性的阶段。这些表型源于MeCP 2的多效性效应，其在神经元祖细胞中非常早地表达并且持续表达至成年。MeCP2的作用是由不同的信号传导，转录和表观遗传机制介导的。逆转Rett综合征影响的尝试需要考虑MeCP2缺失的发育动力学和时间影响。
Rett Syndrome was long considered to be simply a disorder of postnatal development, with phenotypes that manifest only late in development and into adulthood. A variety of recent evidence demonstrates that the phenotypes of Rett Syndrome are present at the earliest stages of brain development, including developmental stages that define neurogenesis, migration, and patterning in addition to stages of synaptic and circuit development and plasticity. These phenotypes arise from the pleotropic effects of MeCP2, which is expressed very early in neuronal progenitors and continues to be expressed into adulthood. The effects of MeCP2 are mediated by diverse signaling, transcriptional, and epigenetic mechanisms. Attempts to reverse the effects of Rett Syndrome need to take into account the developmental dynamics and temporal impact of MeCP2 loss.