A microglial hypothesis of globoid cell leukodystrophy pathology.

A microglial hypothesis of globoid cell leukodystrophy pathology.
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DOI:
10.1002/jnr.23773
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发表时间:
2016-11
影响因子:
4.2
通讯作者:
Crocker, Stephen J.
Crocker, Stephen J.
中科院分区:
医学3区
文献类型:
--
作者:
Nicaise, Alexandra M.;Bongarzone, Ernesto R.;Crocker, Stephen J.

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Globoid cell leukodystrophy (GLD),也被称为Krabbe病,是一种致命的脱髓鞘疾病,伴随着巨大的多核细胞(称为Globoid cells)的形成。这些细胞以前被认为是炎症的副产物,但在疾病早期出现任何损伤迹象之前就可以发现。这些球状细胞引起少突胶质细胞功能障碍的确切机制尚不完全清楚,它们的细胞类型也不明确。在这篇综述中,我们概述了小胶质细胞在GLD中转化为一种未知的、未定义的新型M3表型的观点,这种表型对少突胶质细胞具有细胞毒性,导致疾病进展。
Globoid cell leukodystrophy (GLD), also known as Krabbe 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. In this review we outline 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.
DOI: 10.1097/nen.0000000000000117
发表时间: 2014-10
影响因子: 3.2
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