Article
Altered features of monocytes in adult onset leukoencephalopathy with axonal spheroids and pigmented glia: A clue to the pathomechanism of microglial dyshomeostasis.
Neurobiology of disease - 1 Jul 2020
Hamatani Mio, Yamashita Hirofumi, Ochi Hirofumi, Ashida Shinji, Hashi Yuichiro, Okada Yoichiro, Fujii Chihiro, Kawamura Kazuyuki, Kitazawa Riko, Nakagawa Masanori, Mizuno Toshiki, Takahashi Ryosuke, Kondo Takayuki
Abstract excerpt
Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) is an autosomal-dominant type of leukoencephalopathy caused by gene mutation of colony stimulating factor 1 receptor, which is expressed mainly on monocyte lineage cells such as monocytes in the peripheral blood and microglia in the brain. Hence, microglial dysfunction is regarded as critical in the pathogenesis of ALSP. However,...
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