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球状细胞脑白质营养不良病理学的小胶质细胞假说。

A microglial hypothesis of globoid cell leukodystrophy pathology.

作者信息

Nicaise Alexandra M, Bongarzone Ernesto R, Crocker Stephen J

机构信息

Department of Neuroscience, University of Connecticut School of Medicine, Farmington, Connecticut.

Department of Anatomy and Cell Biology, University of Illinois at Chicago, Chicago, Illinois.

出版信息

J Neurosci Res. 2016 Nov;94(11):1049-61. doi: 10.1002/jnr.23773.

Abstract

Globoid cell leukodystrophy (GLD), also known as Krabbe's disease, is a fatal demyelinating disease accompanied by the formation of giant, multinucleated cells called globoid cells. Previously believed to be a byproduct of inflammation, these cells can be found early in disease before evidence of any damage. The precise mechanism by which these globoid cells cause oligodendrocyte dysfunction is not completely understood, nor is their cell type defined. This Review outlines the idea that microglial cells are transformed into an unknown and undefined novel M3 phenotype in GLD, which is cytotoxic to oligodendrocytes, leading to disease progression. © 2016 Wiley Periodicals, Inc.

摘要

球状细胞脑白质营养不良(GLD),也称为克拉伯病,是一种致命的脱髓鞘疾病,伴有称为球状细胞的巨大多核细胞形成。这些细胞以前被认为是炎症的副产物,在疾病早期,在任何损伤迹象出现之前就能发现它们。球状细胞导致少突胶质细胞功能障碍的确切机制尚未完全了解,其细胞类型也未明确。本综述概述了这样一种观点,即小胶质细胞在GLD中转变为一种未知且未明确的新型M3表型,这种表型对少突胶质细胞具有细胞毒性,导致疾病进展。© 2016威利期刊公司。

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