マスイ　ケンタ   Masui Kenta   増井　憲太    所属   医学部 医学科    職種   准教授
論文種別	原著
言語種別	英語
査読の有無	査読あり
表題	Copper deposition in oligodendroglial cells in an autopsied case of hepatolenticular degeneration.
掲載誌名	正式名：Neuropathology : official journal of the Japanese Society of Neuropathology 略　 称：Neuropathology ISSNコード：(1440-1789)0919-6544(Linking)
掲載区分	国外
巻・号・頁	38(3),pp.321-328
著者・共著者	NISIMUTA Mayu,MASUI Kenta,YAMAMOTO Tomoko,IKARASHI Yuichi,TOKUSHIGE Katsutoshi,HASHIMOTO Etsuko,NAGASHIMA Yoji,SHIBATA Noriyuki
担当区分	2nd著者,責任著者
発行年月	2018/06
概要	We present a case of hepatolenticular degeneration, so-called Wilson's disease (WD), in a 31-year-old Japanese man with broader deposition of copper in the liver, kidney and brain. The liver showed severe cirrhotic changes with macronodular pseudolobule formation, but there was little difference in immunohistochemical expression patterns of the copper transporter ATP7B between the control and present case. In the brain, there were both WD-related lesions such as the scattering of Opalski cells and changes caused by hepatic encephalopathy including the appearance of Alzheimer type II glia. Of note, we identified copper deposits in the systemic organs, including hepatocytes, renal tubules, and in broad areas of the brain. Surprisingly, as a result of further pursuit, copper accumulation in the brain was rarely identified in neuronal cells, but in Olig2-positive glial cells with double immunohistochemical staining. Together, this rare autopsied case suggests a novel cellular candidate affected by abnormal copper metabolism and the necessity to perform the systemic examination of copper deposition in WD.
DOI	10.1111/neup.12456
PMID	29468756