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Present remedies aim at avoiding copper toxicity by chelation, for example, by D-penicillamine (DPA) or bis-choline tetrathiomolybdate (ALXN1840), the latter with a very high copper affinity. Hence, ALXN1840 may potentially avoid neurological deterioration that frequently occurs upon DPA treatment. As the etiology of such worsening is unclear, we reasoned that copper loosely bound to albumin, that is, mimicking a potential liver copper leakage into blood, may damage cells that constitute the blood-brain barrier, which was found to be the case in an <jats:italic>in vitro<\/jats:italic> model using primary porcine brain capillary endothelial cells. Such blood\u2013brain barrier damage was avoided by ALXN1840, plausibly due to firm protein embedding of the chelator bound copper, but not by DPA. Mitochondrial protection was observed, a prerequisite for blood\u2013brain barrier integrity. Thus, high-affinity copper chelators may minimize such deterioration in the treatment of neurologic Wilson disease.<\/jats:p>","DOI":"10.26508\/lsa.202101164","type":"journal-article","created":{"date-parts":[[2021,12,2]],"date-time":"2021-12-02T19:15:27Z","timestamp":1638472527000},"page":"e202101164","update-policy":"https:\/\/doi.org\/10.26508\/lsa.crossmarkpolicy","source":"Crossref","is-referenced-by-count":37,"title":["Bis-choline tetrathiomolybdate prevents copper-induced blood\u2013brain barrier damage"],"prefix":"10.26508","volume":"5","author":[{"given":"Sabine","family":"Borchard","sequence":"first","affiliation":[{"name":"Institute of Molecular Toxicology and Pharmacology, Helmholtz Center Munich, German Research Center for Environmental Health, Neuherberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefanie","family":"Raschke","sequence":"additional","affiliation":[{"name":"Institute of Nutritional Science, University of Potsdam, Nuthetal, 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