Wilson Disease and Other Neurodegenerations with Metal Accumulations

Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F15%3A10294489" target="_blank" >RIV/00216208:11110/15:10294489 - isvavai.cz</a>
Alternative codes found
RIV/00064165:_____/15:10294489
Result on the web
<a href="http://dx.doi.org/10.1016/j.ncl.2014.09.006" target="_blank" >http://dx.doi.org/10.1016/j.ncl.2014.09.006</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1016/j.ncl.2014.09.006" target="_blank" >10.1016/j.ncl.2014.09.006</a>

Result language
angličtina
Original language name
Wilson Disease and Other Neurodegenerations with Metal Accumulations
Original language description
Trace elements, such as iron, copper, manganese, and calcium, which are essential constituents necessary for cellular homeostasis, become toxic when present in excess quantities. In this article, we describe disorders arising from endogenous dysregulation of metal homeostasis leading to their tissue accumulation. Although subgroups of these diseases lead to regional brain metal accumulation, mostly in globus pallidus, which is susceptible to accumulate divalent metal ions, other subgroups cause systemicmetal accumulation affecting the whole brain, liver, and other parenchymal organs. The latter group comprises Wilson disease, manganese transporter deficiency, and aceruloplasminemia and responds favorably to chelation treatment.
Czech name
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Czech description
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Type
J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)
CEP classification
FH - Neurology, neuro-surgery, nuero-sciences
OECD FORD branch
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Project
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Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year
2015
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

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