Poujois, A, Devedjian, JC, Moreau, C et al. (4 more authors) (2016) Bioavailable Trace Metals in Neurological Diseases. Current Treatment Options in Neurology, 18 (10). 46. ISSN 1092-8480
Abstract
Medical treatment in Wilson’s disease includes chelators (d-penicillamine and trientine) or zinc salts that have to be maintain all the lifelong. This pharmacological treatment is categorised into two phases; the first being a de-coppering phase and the second a maintenance one. The best therapeutic approach remains controversial, as only a few non-controlled trials have compared these treatments. During the initial phase, progressive increase of chelators’ doses adjusted to exchangeable copper and urinary copper might help to avoid neurological deterioration. Liver transplantation is indicated in acute fulminant liver failure and decompensated cirrhosis; in cases of neurologic deterioration, it must be individually discussed. During the maintenance phase, the most important challenge is to obtain a good adherence to lifelong medical therapy. Neurodegenerative diseases that lead to a mislocalisation of iron can be caused by a culmination of localised overload (pro-oxidant siderosis) and localised deficiency (metabolic distress). A new therapeutic concept with conservative iron chelation rescues iron-overloaded neurons by scavenging labile iron and, by delivering this chelated metal to endogenous apo-transferrin, allows iron redistribution to avoid systemic loss of iron.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Editors: |
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Copyright, Publisher and Additional Information: | © Springer Science+Business Media, New York 2016. This is an author produced version of a paper published in Current Treatment Options in Neurology . Uploaded in accordance with the publisher's self-archiving policy. The final publication is available at Springer via http://dx.doi.org/10.1007/s11940-016-0426-1 |
Keywords: | Copper; iron; manganese; chelation therapy; reactive species; Neurodegeneration |
Dates: |
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Institution: | The University of Leeds |
Academic Units: | The University of Leeds > Faculty of Biological Sciences (Leeds) > School of Biomedical Sciences (Leeds) |
Funding Information: | Funder Grant number European Commission 633190 - FAIR PARK II - H2020 PHC13-2014/2015 |
Depositing User: | Symplectic Publications |
Date Deposited: | 14 Oct 2016 14:16 |
Last Modified: | 09 Apr 2019 11:03 |
Published Version: | http://dx.doi.org/10.1007/s11940-016-0426-1 |
Status: | Published |
Publisher: | Current Medicine Group |
Identification Number: | 10.1007/s11940-016-0426-1 |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:105987 |