Von Economo neurons are part of a larger neuronal population that are selectively vulnerable in C9orf72 frontotemporal dementia,Neuropathology and Applied Neurobiology

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Von Economo neurons are part of a larger neuronal population that are selectively vulnerable in C9orf72 frontotemporal dementia
Neuropathology and Applied Neurobiology ( IF 4.0 ) Pub Date : 2019-06-10 , DOI: 10.1111/nan.12558
P Gami-Patel ₁ , I van Dijken ₁ , J C van Swieten ₂ , Y A L Pijnenburg ₃ , ₄ , A J M Rozemuller ₁ , J J M Hoozemans ₁ , A A Dijkstra ₁

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The behavioural variant of frontotemporal dementia with a C9orf72 expansion (C9‐bvFTD) is characterised by early changes in social‐emotional cognition that are linked to the loss of von Economo neurons (VENs). Together with a subset of neighbouring pyramidal neurons, VENs express the GABA receptor subunit theta (GABRQ). It is not known if the selective vulnerability of VENs in C9‐bvFTD also includes this GABRQ‐expressing population.

中文翻译：

Von Economo 神经元是更大的神经元群的一部分，在 C9orf72 额颞叶痴呆中选择性脆弱

具有 C9orf72 扩展的额颞叶痴呆的行为变异 (C9-bvFTD) 的特征是社会情绪认知的早期变化，这与 von Economo 神经元 (VEN) 的丧失有关。VEN 与相邻锥体神经元的子集一起表达 GABA 受体亚基 theta (GABRQ)。目前尚不清楚 C9-bvFTD 中 VEN 的选择性脆弱性是否也包括该 GABRQ 表达群体。

更新日期：2019-06-10

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