Characterization of spheroids in hereditary diffuse leukoencephalopathy with axonal spheroids

被引：16

作者：

Jin, Chenghua ^{[1
]}

Washimi, Yukihiko ^{[2
]}

Yoshida, Kunihiro ^{[3
]}

Hashizume, Yoshio ^{[4
]}

Yazawa, Ikuru ^{[1
]}

机构：

[1] NCGG, Lab Res Resources, Res Inst, Obu, Aichi 4748511, Japan

[2] NCGG, Dept Neurol, Obu, Aichi 4748511, Japan

[3] Shinshu Univ, Div Neurogenet, Dept Brain Dis Res, Sch Med, Nagano 3908621, Japan

[4] Fukushimura Hosp, Lab Neuropathol, Toyohashi, Aichi 4418124, Japan

来源：

JOURNAL OF THE NEUROLOGICAL SCIENCES | 2015年 / 352卷 / 1-2期

关键词：

Hereditary diffuse leukoencephalopathy with axonal spheroids; Colony-stimulating factor 1 receptor; Neurodegeneration; Spheroids; White matter degeneration; alpha-Synuclein; MULTIPLE SYSTEM ATROPHY; ALPHA-SYNUCLEIN ACCUMULATION; ADULT-ONSET LEUKODYSTROPHY; NEUROAXONAL SPHEROIDS; NEURONAL DEGENERATION; PIGMENTED GLIA; MOUSE MODEL; DISEASE; ENTITY; HDLS;

D O I：

10.1016/j.jns.2015.03.033

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Hereditary diffuse leukoencephalopathy with axonal spheroids (HDLS) is a neurodegenerative disease clinically characterized by slowly progressive cognitive decline and motor dysfunction. Neuropathology shows diffuse degeneration in the white matter, with prominent presence of widespread axonal spheroids. To investigate the mechanism underlying HDLS neurodegeneration, we characterized spheroids and examined their development in the degenerated white matter. Analysis revealed that the spheroids are an early neuropathological manifestation in the white matter degeneration and involve axonal component proteins and alpha-synudein. The development of spheroids facilitates in initiating neurodegeneration in HDLS. (C) 2015 Elsevier B.V. All rights reserved.

引用

页码：74 / 78

页数：5

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