Adamec E, Chang H T, Stopa E G, Hedreen J C, Vonsattel J P
Department of Psychiatry, Harvard University School of Medicine, McLean Hospital, Belmont, MA 02478, USA.
Neurosci Lett. 2001 Nov 23;315(1-2):21-4. doi: 10.1016/s0304-3940(01)02314-x.
The syndrome of frontotemporal dementia represents a diverse group of diseases presenting with behavioral and cognitive disturbances. The expression of the microtubule-associated protein tau was studied in postmortem samples of frontal cortex of 19 cases (12 Pick's disease A, B, C; 4 dementia lacking distinct histology; 3 motor neuron disease type) by Western blotting with a phosphorylation-independent anti-tau antibody. The presence of tau protein was detected in all cases evaluated, including the 11 brains classified as frontotemporal lobe degeneration (diagnostic categories Pick's disease B, C and dementia lacking distinct histology). These findings indicate that the recently reported decreased expression of tau protein in frontotemporal lobe degeneration represents pathogenic mechanism of neurodegeneration detectable only in a special subset of these disorders.
额颞叶痴呆综合征代表了一组表现为行为和认知障碍的多种疾病。通过使用不依赖磷酸化的抗tau抗体进行蛋白质免疫印迹法,对19例患者(12例匹克病A、B、C型;4例无明显组织学特征的痴呆;3例运动神经元病型)额叶皮质的尸检样本中微管相关蛋白tau的表达进行了研究。在所有评估病例中均检测到tau蛋白的存在,包括11例被归类为额颞叶变性的大脑(诊断类别为匹克病B、C型和无明显组织学特征的痴呆)。这些发现表明,最近报道的额颞叶变性中tau蛋白表达降低代表了仅在这些疾病的一个特殊亚组中可检测到的神经退行性变的致病机制。