Role of Heparin Sulfate in Neuregulin Signaling during Development and in Disease
Role of Heparin Sulfate in Neuregulin Signaling during Development and in Disease
批准号:
6924617
负责人:
MARK S. PANKONIN
金额:
$3.5万
依托单位:
依托单位国家:
美国
项目类别:
财政年份:
2002
资助国家:
美国
项目状态:
已结题
起止时间:
2002-07-01 至 2008-06-30
关键词:
aspartateaxonclinical researchdisease /disorder modelgene expressionhistopathologyhuman subjectimmunocytochemistrylaboratory mouseleukodystrophylight microscopymagnetic resonance imagingmolecular pathologymyelinmyelin proteolipidmyelinopathyneural degenerationneurophysiologynuclear magnetic resonance spectroscopypathologic processpoint mutationtissue /cell culture
中文摘要
轴突丢失是MS患者临床缺陷的重要原因。由于MS的病因尚不清楚,因此不可能确定导致疾病中轴突损伤的事件的确切顺序。对另一种髓鞘相关疾病Pelizaeus- Merzbacher病(PMD)的研究可能会让人们更好地了解替代疗法,这种疾病的病因已知,即影响主要中枢神经系统髓鞘蛋白质脂蛋白(PLP 1)的突变(炎症)PMD中的疾病机制,这在MS中也可能是重要的。磁共振波谱(MRS)和成像(MRI),以及各种组织学技术将被用来确定这种蛋白质的关键结构域,介导其轴突维持功能,试图更好地理解PMD的发病机制和时间进展。希望这些发现将为PMD和MS的临床恶化提供一个模型,并可能导致新的治疗方法。
英文摘要
Axonal loss is an important cause of clinical deficits in patients with MS. Because the etiology of MS is not known, it is impossible to be certain of the precise sequence of events that leads to axonal injury in the disease. The study of another myelin related disorder Pelizaeus- Merzbacher disease (PMD), a disease with a known cause, i.e. mutations affecting the major CNS myelin protein proteolipid protein (PLP1) may provide a better understanding of alternative (to inflammation) disease mechanisms in PMD, which may also be important in MS. Magnetic resonance spectroscopy (MRS), and imaging (MRI), as well as various histological techniques will be utilized to identify the critical domains of this protein that mediate its axon maintaining function in an attempt to better understand the pathogenesis and temporal progression of PMD. Hopefully these findings will provide a model for the clinical deterioration found in PMD as well as MS, and perhaps lead to new therapeutic approaches.
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Role of Heparin Sulfate in Neuregulin Signaling during Development and in Disease
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