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【Neurology】痴呆基因的致病机制
University of Manchester scientists are to investigate the biological causes of the second most common form of dementia after Alzheimer's, thanks to a prestigious £1.9 million senior fellowship award from the Medical Research Council.
Frontotemporal lobar degeneration (FTLD) is a group of dementias that affect the frontal and temporal lobes of the brain and are characterised by behaviour and language dysfunction, rather than the memory loss associated with Alzheimer's disease (AD).
FTLD, which affects about 50,000 people in the UK, also differs from AD in that it targets younger people: FTLD sufferers are usually in their 50s or 60s, although people as young as their 20s have also fallen victim to FTLD. AD sufferers tend to be older.
"Alzheimer's patients lose their awareness of space and time, whereas FTLD can result in changes in personality as well as speech and language difficulties," said Dr Stuart Pickering-Brown, who is leading the research.
"Sufferers can become apathetic or exhibit behaviour at the other extreme and lose normal social values which lead them to act inappropriately.
"Speech and language difficulties fall into two main types: sufferers can develop problems with grammar and pronunciation or have semantic dementia where they lose the information content of language.
Errors in two genes - tau and progranulin - have been identified as causes for FTLD but these only account for 10% of cases. The University of Manchester team plan to investigate the role other genes may play in the disease.
"Our research suggests other genes may be important in regulating the amount of tau and progranulin in the brain," said Dr Pickering-Brown, who is based in the School of Translational Medicine.
"Progranulin is associated with wound healing and little is known about its function in the brain, so we now plan to study the effects of progranulin on cells and explore how it is affected by other genes."
At the end of the five year study, the team hope to have a much clearer understanding of the genetic causes underlying FTLD.
"Understanding the biological problems that lead to FTLD will help in diagnosing the condition and ultimately allow us to find future therapies for patients." 本人已认领该文编译，48小时后若未提交译文，请其他战友自由认 翻译如下，请大家指正！
Study To Reveal Workings Of Dementia Genes
揭示老年痴呆症基因运作的研究
University of Manchester scientists are to investigate the biological causes of the second most common form of dementia after Alzheimer's, thanks to a prestigious £1.9 million senior fellowship award from the Medical Research Council.
英国曼彻斯特大学的科学家们正在研究阿尔茨海默氏症后第二个最常见形式的老年痴呆症的生物原因，其是由医学研究理事会提供的190万英镑高级奖学金。
Frontotemporal lobar degeneration (FTLD) is a group of dementias that affect the frontal and temporal lobes of the brain and are characterised by behaviour and language dysfunction, rather than the memory loss associated with Alzheimer's disease (AD).
额颞叶变性(FTLD)是影响脑中额叶和颞叶的一组痴呆，其特点是行为和语言的障碍，而不是阿尔茨海默病(AD)的记忆丧失。
FTLD, which affects about 50,000 people in the UK, also differs from AD in that it targets younger people: FTLD sufferers are usually in their 50s or 60s, although people as young as their 20s have also fallen victim to FTLD. AD sufferers tend to be older.
FTLD在英国影响到大约50000人，因为它的患者多是比较年轻的人，不同于AD；FTLD患者通常在50岁或60岁，虽然也有20多岁年轻的人患有FTLD。AD患者往往是更年老的人。
"Alzheimer's patients lose their awareness of space and time, whereas FTLD can result in changes in personality as well as speech and language difficulties," said Dr Stuart Pickering-Brown, who is leading the research.
“阿尔茨海默氏症患者失去空间和时间的意识，而FTLD可能会导致人格改变以及言论和语言上的困难，”斯图尔特皮克林布朗博士说，研究组组长。
"Sufferers can become apathetic or exhibit behaviour at the other extreme and lose normal social values which lead them to act inappropriately.
“患者可以变得冷漠或表现出极端的行为，失去正常的社会价值观——导致不当的行为。”
"Speech and language difficulties fall into two main types: sufferers can develop problems with grammar and pronunciation or have semantic dementia where they lose the information content of language.
“言论和语言上的困难分为两大类型：患者可能在语法和发音上出现问题，或具有失去了语言信息含量的词义性痴呆。”
Errors in two genes - tau and progranulin - have been identified as causes for FTLD but these only account for 10% of cases. The University of Manchester team plan to investigate the role other genes may play in the disease.
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作者:admin@医学,生命科学 2010-10-10 05:11
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