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Metallomics Vol - 5 Issue - 4

Metallomics Vol - 5 Issue - 3

Metallomics Vol - 5 Issue - 2

The early molecular processes underlying the neurological manifestations of an animal model of Wilson's disease

Content Provider	Royal Society of Chemistry (RSC)
Author	Kim, Joo Hyun Choi, Jin-Ho Heo, Sun Hee Lee, Beom Hee Kim, Gu-Hwan Yoo, Han-Wook Kim, Jae-Min Kang, Minji
Copyright Year	2013
Abstract	The Long–Evans Cinnamon (LEC) rat shows age-dependent hepatic manifestations that are similar to those of Wilson's disease (WD). The pathogenic process in the brain has, however, not been evaluated in detail due to the rarity of the neurological symptoms. However, copper accumulation is noted in LEC rat brain tissue from 24 weeks of age, which results in oxidative injuries. The current study investigated the gene expression profiles of LEC rat brains at 24 weeks of age in order to identify the important early molecular changes that underlie the development of neurological symptoms in WD. Biological ontology-based analysis revealed diverse altered expressions of the genes related to copper accumulation. Of particular interest, we found altered expression of genes connected to mitochondrial respiration (Sdhaf2 and Ndufb7), calcineurin-mediated cellular processes (Ppp3ca, Ppp3cb, and Camk2a), amyloid precursor protein (Anks1b and A2m) and alpha-synuclein (Snca). In addition to copper-related changes, compensatory upregulations of Cp and Hamp reflect iron-mediated neurotoxicity. Of note, reciprocal expression of Asmt and Bhmt is an important clue that altered S-adenosylhomocysteine metabolism underlies brain injury in WD, which is directly correlated to the decreased expression of S-adenosylhomocysteine hydrolase in hepatic tissue in LEC rats. In conclusion, our study indicates that diverse molecular changes, both variable and complex, underlie the development of neurological manifestations in WD. Copper-related injuries were found to be the principal pathogenic process, but Fe- or adenosylhomocysteine-related injuries were also implicated. Investigations using other animal models or accessible human samples will be required to confirm our observations.
Starting Page	532
Ending Page	540
Page Count	9
File Format	HTM / HTML PDF
ISSN	17565901
Volume Number	5
Issue Number	5
Journal	Metallomics
DOI	10.1039/c3mt20243g
Language	English
Publisher	Royal Society of Chemistry
Access Restriction	Open
Subject Keyword	Hydrolase Evans Cinnamon Asmt Wilson LEC Metabolism Amyloid precursor protein 24 Weeks Neurotoxicity Gene Cellular respiration Copper S-Adenosyl-L-homocysteine Hamp Alpha-synuclein
Content Type	Text
Resource Type	Article
Subject	Chemistry Medicine Metals and Alloys Biochemistry Biomaterials Biophysics

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The early molecular processes underlying the neurological manifestations of an animal model of Wilson's disease

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The early molecular processes underlying the neurological manifestations of an animal model of Wilson's disease