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  科学家已识别出ALS的敏感特异生化标志物
   
  据路透社2004年4月19日纽约讯

摘要:
肌萎缩侧索硬化(ALS)是一种肌肉萎缩性疾病,通常在病人经历数月症状后才能检测出来。它能引起进行性麻痹,但是早期症状不明显。星期一,美国研究员发现一种能快速诊断ALS的方法。但是,仍需进行大量试验以确定其有效性。

科学家第一次鉴定出了可敏感、特异地指示ALS发作的蛋白质生化标志物。美国匹兹堡大学的布塞(Robert P. Bowser)告诉路透社记者,最终可能根据这组蛋白质形成一种迅速诊断ALS的检测方法。

"通常,诊断ALS需要一年",布塞解释,"而现在我们可能通过一滴脑脊液(CSF)在一天内做到"。早期诊断非常重要,因为FDA批准的治疗ALS的药物只有在症状一发作就开始使用才能发挥最大作用。

布塞的研究小组检查了25名新诊断为ALS的病人和35名对照者(其中部分人有其它与ALS相似的神经疾病,如肌肉无力或运动功能丧失)CSF中的ALS生化标志物。"CSF与ALS可能影响的胶质和神经细胞接触密切,因而是分析蛋白质变化的理想介质",作者在美国研究性病理学会的"实验性生物学2004"上解释。因此他们使用质谱分析技术找到了能区别出两组参试者的10种生化标志物,其蛋白质最大浓度在两组之间有显著性差异,对ALS有92%的敏感性和79%的特异性。

现在这些研究者正在对这组蛋白质进行排序,下一步他们将在更多病人中检测这些假定ALS标志物的价值。另外,他们对研究该蛋白质峰值随着ALS进展而变化的模式也很有兴趣。


周边细胞救助ALS损坏的神经元
新的发现指出,在遗传性Lou Gehrig 症的一个小鼠模式中,附近的细胞能够加快或减慢运动神经元的损坏。Lou Gehrig 症也称ALS, 是一种进行性的神经退化症,患者的运动神经元在他们中年时开始死亡。A.M. Clement和一个美国和加拿大的小组集中研究了一种罕见家族ALS,这与SOD1基因的变异有关。科学家们制造出一些小鼠,它们带有一些正常的细胞,也带有能够表达变异的人类SOD1蛋白的基因。他们发现健康的环境能够"救援"带有变异的运动神经元。相反,附近的不正常细胞也能损坏健康的运动神经元。作者们做出结论?quot;运动神经元的死亡在原理上能唯一被周边多种细胞的破坏激起。"
美发现快速检测ALS的方法


WASHINGTON (Reuters) -- U.S. researchers said Monday they had found a quicker way to diagnose a paralyzing muscle disease called ALS, perhaps in time for drugs to delay its fatal progression.

ALS,or Lou Gehrig's disease, is usually diagnosed after a patient has been suffering from its symptoms for months. ALS, part of a group of diseases called Motor Neuron Disease, causes progressive paralysis but the symptoms are not clear at first.

A team at the University of Pittsburgh said they had identified a kind of protein fingerprint, called a biomarker, that can tell right away that someone has the deadly and incurable condition.

One drug, Rilutek, known generically as riluzole, is approved for use in treating ALS patients and some studies have suggested it works best when used early. Dr.Robert Bowser and colleagues examined the CSF of 25 patients recently diagnosed with ALS and 35 people without ALS.

Some of ALS-free "controls had other neurological diseases that looked something like ALS, including muscle weakness and loss of motor function.
Bowser's team used protein chips and computer programs to identify a pattern of protein activity that was unique to the ALS patients.

Proteins are produced by cells based on genetic instructions and perform all the body's functions.
The 10 proteins and their pattern of production could make for an easy ALS test that could give an answer within a few hours, Bowser told a meeting of the American Physiological Society, part of a larger conference in Washington called Experimental Biology 2004.

The new assay needs to be given to larger groups, while separate tests also need to be carried out to see if it can predict who, with early symptoms, has ALS before they are formally diagnosed.
Bowser's team is also working to find out what each of the proteins does, specifically, in cells.
ALS affects an estimated 100,000 people worldwide and at least 5,000 people are diagnosed every year in the United States. The baseball player Lou Gehrig, with whom ALS is most commonly associated, died of the disease in 1941.

   
             
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