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培养的成纤维细胞模型。

The cultured fibroblast model.

作者信息

Blass J P

机构信息

Altschul Laboratory for Dementia Research, Burke Medical Research Institute, Cornell University Medical College, White Plains, New York.

出版信息

J Neural Transm Suppl. 1994;44:87-95. doi: 10.1007/978-3-7091-9350-1_7.

Abstract

Cultured skin fibroblasts have been successfully used to elucidate the molecular and biochemical bases of a large number of inborn errors of metabolism which cause neurological disease. The fibroblast model is now being extended to studies of genetic diseases of the nervous system with later clinical onset, including Alzheimer's Disease (AD). Persistence in the cultured cells of an abnormality found in AD brain provides evidence that the abnormality in brain is not simply a reflection of anatomic or chemical damage in the clinically affected tissue. Biochemical and cell biological studies of AD fibroblasts are succeeding in implicating molecules which warrant further study at the molecular genetic level. In neurological diseases with an important genetic component, including AD, studies of fibroblasts can be a useful adjunct to studies of brain.

摘要

培养的皮肤成纤维细胞已成功用于阐明大量导致神经疾病的先天性代谢缺陷的分子和生化基础。成纤维细胞模型目前正在扩展到对神经系统晚发性遗传疾病的研究,包括阿尔茨海默病(AD)。AD大脑中发现的异常在培养细胞中的持续存在提供了证据,表明大脑中的异常不仅仅是临床受影响组织中解剖或化学损伤的反映。对AD成纤维细胞的生化和细胞生物学研究成功地发现了一些分子,这些分子值得在分子遗传学水平上进一步研究。在包括AD在内的具有重要遗传成分的神经疾病中,对成纤维细胞的研究可以成为大脑研究的有用辅助手段。

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