Wilson disease and other neurodegenerations with metal accumulations
Jazyk angličtina Země Spojené státy americké Médium print
Typ dokumentu časopisecké články, přehledy, audiovizuální média
PubMed
25432729
DOI
10.1016/j.ncl.2014.09.006
PII: S0733-8619(14)00074-7
Knihovny.cz E-zdroje
- Klíčová slova
- Chelating therapy, Manganism, NBIA, Neurodegeneration with brain iron accumulation, Primary familial brain calcification, Wilson disease,
- MeSH
- chelátová terapie MeSH
- duševní poruchy etiologie MeSH
- hepatolentikulární degenerace epidemiologie etiologie patofyziologie terapie MeSH
- kovy metabolismus toxicita MeSH
- lidé MeSH
- mozek metabolismus MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- audiovizuální média MeSH
- časopisecké články MeSH
- přehledy MeSH
- Názvy látek
- kovy MeSH
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 systemic metal 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.
Citace poskytuje Crossref.org
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