Angelman syndrome (AS) is a debilitating neurogenetic disorder characterized by severe developmental delay, speech impairment, gait ataxia, sleep disturbances, epilepsy, and a unique behavioral phenotype. AS is caused by a microdeletion or mutation in the maternal 15q11-q13 chromosome region containing UBE3A gene. The hippocampus is one of the important brain regions affected in AS mice leading to substantial hippocampal-dependent cognitive and behavioral deficits. Recent studies have suggested an abnormal increase in the α1-Na/K-ATPase (α1-NaKA) in AS mice as the precipitating factor leading to the hippocampal deficits. A subsequent study showed that the hippocampal-dependent behavioral deficits occur as a result of altered calcium (Ca+2) dynamics in the CA1 pyramidal neurons (PNs) caused by the elevated α1-NaKA expression levels in the AS mice. Nonetheless, a causal link between hippocampal deficits and major behavioral phenotypes in AS is still obscure. Subiculum, a region adjacent to the hippocampal CA1 is the major output source of the hippocampus and plays an important role in the transfer of information from the CA1 region to the cortical areas. However, in spite of the robust hippocampal deficits and several known electrophysiological alterations in multiple brain regions in AS mice, the neuronal properties of the subicular neurons were never investigated in these mice. Additionally, subicular function is also implied in many neuropsychiatric disorders such as autism, schizophrenia, Alzheimer's disease, and epilepsy that share some common features with AS. Therefore, given the importance of the subiculum in these neuropsychiatric disorders and the altered electrophysiological properties of the hippocampal CA1 PNs projecting to the subiculum, we sought to examine the subicular PNs. We performed whole-cell recordings from dorsal subiculum of both WT and AS mice and found three distinct populations of PNs based on their ability to fire bursts or single action potentials following somatic current injection: strong bursting, weak bursting, and regular firing neurons. We found no overall differences in the distribution of these different subicular PN populations among AS and WT controls. However, the different cell types showed distinct alterations in their intrinsic membrane properties. Further, none of these populations were altered in their excitatory synaptic properties. Altogether, our study characterized the different subtypes of PNs in the subicular region of an AS mouse model.

中文翻译：

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Angelman 综合征小鼠模型中背下托的规则和突发放电锥体神经元的电生理特征。

Angelman 综合征 (AS) 是一种使人衰弱的神经遗传性疾病，其特征是严重的发育迟缓、语言障碍、步态共济失调、睡眠障碍、癫痫和独特的行为表型。AS 是由含有 UBE​​3A 基因的母体 15q11-q13 染色体区域的微缺失或突变引起的。海马体是 AS 小鼠中受影响的重要大脑区域之一，导致大量依赖海马体的认知和行为缺陷。最近的研究表明，AS 小鼠中 α1-Na/K-ATP 酶 (α1-NaKA) 的异常增加是导致海马缺陷的诱发因素。随后的一项研究表明，海马依赖性行为缺陷的发生是由于 AS 小鼠中 α1-NaKA 表达水平升高导致 CA1 锥体神经元 (PN) 中钙 (Ca+2) 动力学改变的结果。尽管如此，海马缺陷与 AS 中主要行为表型之间的因果关系仍不清楚。Subiculum 是海马 CA1 附近的一个区域，是海马的主要输出源，在从 CA1 区域到皮层区域的信息传递中起着重要作用。然而，尽管 AS 小鼠的多个大脑区域存在明显的海马缺陷和几种已知的电生理改变，但从未在这些小鼠中研究过亚突神经元的神经元特性。此外，许多神经精神疾病如自闭症、精神分裂症、阿尔茨海默病和癫痫也暗示了亚突功能，这些疾病与 AS 有一些共同的特征。所以，鉴于下突在这些神经精神疾病中的重要性以及投射到下突的海马 CA1 PN 的电生理特性改变，我们试图检查下突 PN。我们对 WT 和 AS 小鼠的背下下叶进行了全细胞记录，并根据它们在体细胞电流注射后激发爆发或单一动作电位的能力发现了三个不同的 PN 群体：强爆发、弱爆发和规则激发神经元。我们发现 AS 和 WT 对照中这些不同的皮下 PN 种群的分布没有总体差异。然而，不同的细胞类型在其固有的膜特性方面表现出不同的改变。此外，这些种群的兴奋性突触特性均未发生改变。共，