Fibrodysplasia ossificans progressiva: histopathological implications of aberrant BMP signalling for CNS dysgenesis.
Fibrodysplasia ossificans progressiva: histopathological implications of aberrant BMP signalling for CNS dysgenesis.
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进行性骨化性纤维发育不良：异常 BMP 信号传导对中枢神经系统发育不全的组织病理学影响。
DOI：
10.1111/nan.12805
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发表时间：
2022
影响因子：
5
通讯作者：
and Akiyoshi Kakita
中科院分区：
文献类型：
--
作者：
Hidetomo Tanaka;Hiroshi Shimizu;Yosuke Yonemochi;Tetsuo Ozawa;Yasuko Toyoshima;Takashi Nakajima;and Akiyoshi Kakita
FOP 脑干病变中观察到的神经元与 FOP 和对照脑干核中观察到的神经元的比较。(A, B) 本患者 (A) 和对照受试者 (B) 的上橄榄核都含有形态相似的新月形神经元。此外，在一名 I ACVR1 i 杂合性 G356D 突变的尸检患者中[8]，构成上橄榄核和延髓背侧病变的细胞分别是星形胶质细胞和室管膜细胞——与本例患者的情况不同。关键词：ACVR1； BMP 信号传导；中枢神经系统组织发生；进行性骨化性纤维发育不良;室下区 EN ACVR1 BMP 信号传导 中枢神经系统组织发生 进行性骨化性纤维发育不良 室下区 1 6 6 05/10/22 20220601 NES 220601 要点 已知骨形态发生蛋白 (BMP) 信号传导与软骨形成和中枢神经系统组织发生有关，尽管后者机制的具体细节仍不清楚。[摘自文章]版权所有《神经病理学与应用神经生物学》的版权属于 Wiley-Blackwell 的财产，未经版权所有者明确书面许可，不得将其内容复制或通过电子邮件发送到多个网站或发布到列表服务器。但是，用户可以打印、下载或通过电子邮件发送文章供个人使用。本摘要可能会被删节。不保证副本的准确性。用户应参阅该材料的原始发布版本以获取完整摘要。版权适用于所有摘要。
Comparisons of the neurons observed in the brainstem lesions in FOP and those in the brainstem nuclei in FOP and controls.(A, B) The superior olivary nucleus in both the present patient (A) and control subject (B) contains morphologically similar crescent-shaped neurons. Furthermore, in an autopsied patient with a heterozygous G356D mutation in I ACVR1 i [8], the cells composing the superior olivary nuclei and dorsal medulla oblongata lesions were astrocytes and ependymal cells, respectively-unlike the situation in the present patient. Keywords: ACVR1; BMP signalling; CNS histogenesis; fibrodysplasia ossificans progressiva; subventricular zone EN ACVR1 BMP signalling CNS histogenesis fibrodysplasia ossificans progressiva subventricular zone 1 6 6 05/10/22 20220601 NES 220601 Key points Bone morphogenetic protein (BMP) signalling is known to be associated with chondrogenesis and CNS histogenesis, although precise details of the latter mechanism remain unknown.[Extracted from the article]Copyright of Neuropathology & Applied Neurobiology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. Copyright applies to all Abstracts.