Phenylketonuria-related synaptic changes in a BTBR-Pahenu2 mouse model
Phenylketonuria-related synaptic changes in a BTBR-Pahenu2 mouse model
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DOI：
10.1097/wnr.0b013e3283495acc
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发表时间：
2011-08-24
期刊：
影响因子：
1.7
通讯作者：
Zhang, Xinshun
中科院分区：
文献类型：
--
作者：
Liang, Lili;Gu, Xuefan;Zhang, Xinshun
苯丙酮尿症是最常见的遗传性氨基酸病，与脑损伤有关。到目前为止，还没有关于苯丙酮尿症遗传小鼠模型BTBR-PAH(Enu2)的神经病理学的研究。我们研究了野生型、杂合型和BTBR-PAH(Enu2)小鼠在CA1和前额叶皮质中的树突棘和突触。在BTBR-PAH(Enu2)小鼠中，树突棘密度降低，突触前活动区长度缩短，突触间隙变宽，突触后密度降低。同时，在BTBR-PAH(Enu2)小鼠中，钙/钙调蛋白依赖的蛋白激酶IIα在Thr286处的磷酸化被警示。这些发现揭示了苯丙酮尿症相关的脑损伤伴随着树突棘和突触的异常。钙/钙调蛋白依赖的蛋白激酶IIα功能障碍可能导致突触功能受损。神经报告22：617-622(C)2011 Wolters Kluwer Health竖条Lippincott Williams&Wilkins。
Phenylketonuria is the most common, inherited aminoacidopathy associated with brain injury. To date, no study has focused on the neuropathology of the genetic mouse model of phenylketonuria, BTBR-Pah(enu2). We examined dendritic spines and synapses in the CA1 and prefrontal cortex among the wild-type, heterozygous, and BTBR-Pah(enu2) mice. A reduced density of dendritic spines, a shortened length of the presynaptic active zone, a widened synaptic cleft, and decreased thickness of postsynaptic density were revealed in BTBR-Pah(enu2) mice. Meanwhile, the phosphorylation at Thr286 of Ca2+/calmodulin-dependent protein kinase II alpha was alerted in BTBR-Pah(enu2) mice. These findings revealed that phenylketonuria-related brain impairment is accompanied with abnormalities of dendritic spines and synapses. The dysfunction of Ca2+/calmodulin-dependent protein kinase II alpha may result in an impaired synaptic function. NeuroReport 22:617-622 (C) 2011 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins.