Nishimura I, Uetsuki T, Dani S U, Ohsawa Y, Saito I, Okamura H, Uchiyama Y, Yoshikawa K
Division of Regulation of Macromolecular Functions, Institute for Protein Research, Osaka University, Suita, Osaka 565, Japan.
J Neurosci. 1998 Apr 1;18(7):2387-98. doi: 10.1523/JNEUROSCI.18-07-02387.1998.
In an attempt to elucidate the pathological implications of intracellular accumulation of the amyloid precursor protein (APP) in postmitotic neurons in vivo, we transferred APP695 cDNA into rat hippocampal neurons by using a replication-defective adenovirus vector. We first improved the efficiency of adenovirus-mediated gene transfer into neurons in vivo by using hypertonic mannitol. When a beta-galactosidase-expressing recombinant adenovirus suspended in 1 M mannitol was injected into a dorsal hippocampal region, a number of neurons in remote areas were positively stained, presumably owing to increased retrograde transport of the virus. When an APP695-expressing adenovirus was injected into the same site, part of the infected neurons in the hippocampal formation underwent severe degeneration in a few days, whereas astrocytes near the injection site showed no apparent degeneration. These degenerating neurons accumulated different epitopes of APP, and beta/A4 protein (Abeta)-immunoreactive materials were undetected in the extracellular space. A small number of degenerating neurons showed nuclear DNA fragmentation. Electron microscopic examinations demonstrated that degenerating neurons had shrunken perikarya along with synaptic abnormalities. Microglial cells/macrophages were often found in close proximity to degenerating neurons, and in some cases they phagocytosed these neurons. These results suggest that intracellular accumulation of wild-type APP695 causes a specific type of neuronal degeneration in vivo in the absence of extracellular Abeta deposition.
为了阐明淀粉样前体蛋白(APP)在有丝分裂后神经元内的细胞内积累在体内的病理意义,我们使用复制缺陷型腺病毒载体将APP695 cDNA转入大鼠海马神经元。我们首先通过使用高渗甘露醇提高了腺病毒介导的基因在体内向神经元转移的效率。当将悬浮于1 M甘露醇中的表达β-半乳糖苷酶的重组腺病毒注射到背侧海马区时,远处区域的许多神经元被阳性染色,推测是由于病毒逆行运输增加所致。当将表达APP695的腺病毒注射到同一部位时,海马结构中部分被感染的神经元在数天内发生严重退变,而注射部位附近的星形胶质细胞未显示明显退变。这些退变的神经元积累了APP的不同表位,并且在细胞外空间未检测到β/A4蛋白(Aβ)免疫反应性物质。少数退变神经元显示核DNA片段化。电子显微镜检查表明,退变神经元的胞体缩小并伴有突触异常。经常发现小胶质细胞/巨噬细胞紧邻退变神经元,在某些情况下它们吞噬这些神经元。这些结果表明,在没有细胞外Aβ沉积的情况下,野生型APP695的细胞内积累在体内导致特定类型的神经元退变。