Lin Wen-Lang, Lewis Jada, Yen Shu-Hui, Hutton Michael, Dickson Dennis W
Mayo Clinic, Jacksonville, Florida, USA.
Am J Pathol. 2003 Jan;162(1):213-8. doi: 10.1016/S0002-9440(10)63812-6.
We recently reported a transgenic mouse line (JNPL3) that expresses mutant (P301L) tau and develops neurofibrillary tangles composed of filamentous tau aggregates. Here we show that these mice have abnormal tau filaments not only in neurons, but also in oligodendrocytes and astrocytes. Similar results were detected in another transgenic line (JNPL2+3+) that expresses the longest human tau isoform with the P301L mutation. The ultrastructure of the tau filaments and immunoreactivity with tau and ubiquitin antibodies were similar in glia and neurons. Given similarities of the lesions in the mice to human neuronal and glial inclusions, these transgenic mice appear to be a valuable model to study pathogenesis of the neurodegenerative tauopathies.
我们最近报道了一种转基因小鼠品系（JNPL3），其表达突变型（P301L）tau蛋白，并形成由丝状tau聚集体组成的神经原纤维缠结。在此我们表明，这些小鼠不仅在神经元中，而且在少突胶质细胞和星形胶质细胞中都有异常的tau细丝。在另一种表达具有P301L突变的最长人类tau异构体的转基因品系（JNPL2+3+）中也检测到了类似结果。tau细丝的超微结构以及与tau和泛素抗体的免疫反应性在神经胶质细胞和神经元中相似。鉴于小鼠病变与人类神经元和神经胶质细胞包涵体的相似性，这些转基因小鼠似乎是研究神经退行性tau蛋白病发病机制的有价值模型。
