title: Characterization of spheroids in hereditary diffuse leukoencephalopathy with axonal spheroids.
abstract: 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 α-synuclein. The development of spheroids facilitates in initiating neurodegeneration in HDLS.
authors.affiliation: Laboratory of Research Resources, Research Institute, National Center for Geriatrics and Gerontology, Aichi 474-8511, Japan.
authors.forename: Chenghua
authors.initials: C
authors.lastname: Jin
authors.affiliation: Department of Neurology, National Center for Geriatrics and Gerontology, Aichi 474-8511, Japan.
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Washimi
authors.affiliation: Division of Neurogenetics, Department of Brain Disease Research, Shinshu University School of Medicine, Nagano 390-8621, Japan.
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Yoshida
authors.affiliation: Laboratory of Neuropathology, Fukushimura Hospital, Aichi 441-8124, Japan.
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Hashizume
authors.affiliation: Laboratory of Research Resources, Research Institute, National Center for Geriatrics and Gerontology, Aichi 474-8511, Japan. Electronic address: yazawaik@ncgg.go.jp.
authors.forename: Ikuru
authors.initials: I
authors.lastname: Yazawa
mesh_terms: D000328:Adult; D001369:Axons / Q000473:pathology; D005260:Female; D006801:Humans; D056784:Leukoencephalopathies / Q000235:genetics / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D019636:Neurodegenerative Diseases / Q000235:genetics / Q000473:pathology*; D016186:Receptor, Macrophage Colony-Stimulating Factor / Q000235:genetics; D066127:White Matter / Q000473:pathology*; D051844:alpha-Synuclein / Q000235:genetics
chemical_list: D051844:alpha-Synuclein; D016186:Receptor, Macrophage Colony-Stimulating Factor
keywords: Colony-stimulating factor 1 receptor; Hereditary diffuse leukoencephalopathy with axonal spheroids; Neurodegeneration; Spheroids; White matter degeneration; α-Synuclein