Copper deposition in oligodendroglial cells in an autopsied case of hepatolenticular degeneration

Neuropathology : Official Journal of the Japanese Society of Neuropathology
Mayu NishimutaNoriyuki Shibata

Abstract

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.

References

Nov 30, 1993·Biochemical and Biophysical Research Communications·Y YamaguchiJ D Gitlin
Sep 10, 2003·Mayo Clinic Proceedings·Mounif El-Youssef
Feb 6, 2007·Lancet·Aftab AlaMichael L Schilsky
Feb 5, 2008·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·S Meenakshi-SundaramS K Shankar
May 29, 2008·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Eve A RobertsUNKNOWN American Association for Study of Liver Diseases (AASLD)
Dec 2, 2010·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Kenta MasuiToru Iwaki
May 28, 2011·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Kenta MasuiToru Iwaki
Jan 14, 2012·Gastroenterology·Dominik HusterSvetlana Lutsenko
Aug 30, 2013·Frontiers in Aging Neuroscience·Jonathon TelianidisSharon La Fontaine
Oct 15, 2013·Revue neurologique·J-M TrocelloF Woimant
Dec 17, 2014·Lancet Neurology·Oliver BandmannStephen G Kaler
Mar 25, 2015·International Journal of Molecular Sciences·Fei WuChunmeng Jiang
Jul 25, 2015·Annals of Human Biology·Amanda Gomes, George V Dedoussis
Dec 23, 2015·World Journal of Hepatology·Kryssia Isabel Rodriguez-CastroGiacomo Carlo Sturniolo
Apr 24, 2017·Handbook of Clinical Neurology·Peter Ferenci
Apr 24, 2017·Handbook of Clinical Neurology·Irene J Chang, Si Houn Hahn
Apr 24, 2017·Handbook of Clinical Neurology·Aurélia PoujoisFrance Woimant

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Citations

Dec 10, 2020·Metallomics : Integrated Biometal Science·Ashima BhattacharjeeKaustav Chakraborty

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