研究人員從阿爾茨海默氏癥患者大腦中提取出纖維狀糾纏物,，當(dāng)他們將這些糾纏物注入健康小鼠的大腦后,，小鼠的大腦中也形成了類(lèi)似的糾纏物，新成果發(fā)表在日前在線出版的《自然—細(xì)胞生物學(xué)》期刊上,。新發(fā)現(xiàn)表明,，這種糾纏物有傳染性，而且可能與朊病毒類(lèi)似,，朊病毒與傳染性腦疾病如瘋牛病和人類(lèi)庫(kù)茲德-賈克氏病等相關(guān),。
微管相關(guān)蛋白是阿爾茨海默氏癥患者大腦神經(jīng)元纖維纏結(jié)的組成部分。Markus Tolnay和同事從表達(dá)一種變異人類(lèi)tau蛋白質(zhì)的小鼠大腦中提取出部分腦組織,，并將這些大腦提取物注入普通小鼠的大腦特定區(qū)域,。他們發(fā)現(xiàn)，這些大腦提取物引導(dǎo)人類(lèi)tau蛋白質(zhì)在普通小鼠大腦內(nèi)聚集,，形成神經(jīng)元纖維纏結(jié),。此外，這些新形成的纏結(jié)擴(kuò)散到了大腦中的附近區(qū)域,。
以前,，研究人員在tau蛋白疾病家族中神經(jīng)退化性疾病患者的體內(nèi)觀察到了tau包含物的形成，但認(rèn)為它們沒(méi)有傳染性,。而朊蛋白質(zhì)則被認(rèn)為具有傳染性,，它們因折疊成非正常結(jié)構(gòu)而聚集在一起，這樣,，它們就能將正常的配對(duì)物轉(zhuǎn)化為類(lèi)似的非正常結(jié)構(gòu),。（生物谷Bioon.com）
生物谷推薦原始出處：
Nature Cell Biology 11, 909 - 913 (2009)7 June 2009 | doi:10.1038/ncb1901
Transmission and spreading of tauopathy in transgenic mouse brain
Florence Clavaguera1, Tristan Bolmont2, R. Anthony Crowther3, Dorothee Abramowski4, Stephan Frank1, Alphonse Probst1, Graham Fraser3, Anna K. Stalder5, Martin Beibel4, Matthias Staufenbiel4, Mathias Jucker2, Michel Goedert3,6 & Markus Tolnay1,6
Hyperphosphorylated tau makes up the filamentous intracellular inclusions of several neurodegenerative diseases, including Alzheimer's disease1. In the disease process, neuronal tau inclusions first appear in the transentorhinal cortex from where they seem to spread to the hippocampal formation and neocortex2. Cognitive impairment becomes manifest when inclusions reach the hippocampus, with abundant neocortical tau inclusions and extracellular -amyloid deposits being the defining pathological hallmarks of Alzheimer's disease. An abundance of tau inclusions, in the absence of -amyloid deposits, defines Pick's disease, progressive supranuclear palsy, corticobasal degeneration and other diseases1. Tau mutations cause familial forms of frontotemporal dementia, establishing that tau protein dysfunction is sufficient to cause neurodegeneration and dementia3, 4, 5. Thus, transgenic mice expressing mutant (for example, P301S) human tau in nerve cells show the essential features of tauopathies, including neurodegeneration and abundant filaments made of hyperphosphorylated tau protein6, 8. By contrast, mouse lines expressing single isoforms of wild-type human tau do not produce tau filaments or show neurodegeneration7, 8. Here we have used tau-expressing lines to investigate whether experimental tauopathy can be transmitted. We show that injection of brain extract from mutant P301S tau-expressing mice into the brain of transgenic wild-type tau-expressing animals induces assembly of wild-type human tau into filaments and spreading of pathology from the site of injection to neighbouring brain regions.
1 Department of Neuropathology, Institute of Pathology, University of Basel, Basel, Switzerland.
2 Department of Cellular Neurology, Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.
3 MRC Laboratory of Molecular Biology, Cambridge, UK.
4 Novartis Institutes for Biomedical Research, Basel, Switzerland.
5 Neurology and Neurobiology, University Hospital, Basel, Switzerland.
6 These authors contributed equally to this work
