Department of Psychiatry and Neurosciences, Rashid Laboratory for Developmental Neurobiology, Morsani College of Medicine, University of South Florida, Tampa, Florida 33613, USA.
Glia. 2013 Sep;61(9):1556-69. doi: 10.1002/glia.22544. Epub 2013 Jul 10.
Autistic individuals display impaired social interactions and language, and restricted, stereotyped behaviors. Elevated levels of secreted amyloid precursor protein-alpha (sAPPα), the product of α-secretase cleavage of APP, are found in the plasma of some individuals with autism. The sAPPα protein is neurotrophic and neuroprotective and recently showed a correlation to glial differentiation in human neural stem cells (NSCs) via the IL-6 pathway. Considering evidence of gliosis in postmortem autistic brains, we hypothesized that subsets of patients with autism would exhibit elevations in CNS sAPPα and mice generated to mimic this observation would display markers suggestive of gliosis and autism-like behavior. Elevations in sAPPα levels were observed in brains of autistic patients compared to controls. Transgenic mice engineered to overexpress human sAPPα (TgsAPPα mice) displayed hypoactivity, impaired sociability, increased brain glial fibrillary acidic protein (GFAP) expression, and altered Notch1 and IL-6 levels. NSCs isolated from TgsAPPα mice, and those derived from wild-type mice treated with sAPPα, displayed suppressed β-tubulin III and elevated GFAP expression. These results suggest that elevations in brain sAPPα levels are observed in subsets of individuals with autism and TgsAPPα mice display signs suggestive of gliosis and behavioral impairment.
自闭症个体表现出社交互动和语言障碍,以及受限和刻板的行为。一些自闭症患者的血浆中发现了高水平的分泌型淀粉样前体蛋白-α(sAPPα),这是 APP 的α-分泌酶切割的产物。sAPPα 蛋白具有神经营养和神经保护作用,最近通过 IL-6 途径显示与人类神经干细胞(NSC)的神经胶质分化相关。考虑到自闭症患者死后大脑中存在神经胶质增生的证据,我们假设自闭症患者的亚组会表现出中枢神经系统 sAPPα 的升高,并且模拟这种观察结果的小鼠会表现出神经胶质增生和自闭症样行为的标志物。与对照组相比,自闭症患者的大脑中观察到 sAPPα 水平升高。过表达人 sAPPα 的转基因小鼠(TgsAPPα 小鼠)表现出活动减少、社交能力受损、大脑神经胶质纤维酸性蛋白(GFAP)表达增加以及 Notch1 和 IL-6 水平改变。从 TgsAPPα 小鼠中分离出的 NSCs,以及用 sAPPα 处理的野生型小鼠衍生的 NSCs,表现出 β-微管蛋白 III 表达下调和 GFAP 表达上调。这些结果表明,自闭症患者的亚组中观察到大脑 sAPPα 水平升高,TgsAPPα 小鼠表现出神经胶质增生和行为障碍的迹象。