Spontaneous hepatic copper accumulation in Long-Evans Cinnamon rats with hereditary hepatitis. A model of Wilson's disease.

Eleven Bedlington terriers were found to have a mean hepatic copper concentration of 6,321 micrograms/g dry wt (normal, 200 micrograms/g dry wt) and renal copper concentration that was three or four times normal. Brain copper levels were normal in younger dogs, were elevated in two older dogs, and were 100 times normal in one dog that died of the disease. Increased concentrations of copper in the liver, kidney, and brain also characterize Wilson's disease. Erythrocyte survival was normal in three affected dogs, but serum glutamic-pyruvic transaminase levels were usually elevated. Unlike the hypoceruloplasminemia of patients with Wilson's disease, plasma ceruloplasmin activity was not only normal but was also slightly elevated in the terriers. Despite their normal or excessive ceruloplasmin, the Bedlington terriers could convert ionic 64Cu to radioceruloplasmin but did so only very slowly. These dogs accumulated significantly more 64Cu in their livers than normal, much like patients with Wilson's disease do before symptoms develop.

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Mechanism of hepatorenal syndrome in rats of Long–Evans Cinnamon strain, an animal model of fulminant Wilson's disease

Toxicology ◽

10.1016/s0300-483x(99)00005-0 ◽

1999 ◽

Vol 132 (2-3) ◽

pp. 201-214 ◽

Cited By ~ 14

Author(s):

Kazuo Nomiyama ◽

Hiroko Nomiyama ◽

Naoki Kameda ◽

Akihiro Tsuji ◽

Hiromu Sakurai

Keyword(s):

Animal Model ◽

Hepatorenal Syndrome ◽

Wilson’S Disease ◽

Wilson's Disease ◽

Long Evans

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Increased abundance of GABAA receptor subunit mRNAs in the brain of Long–Evans Cinnamon rats, an animal model of Wilson's disease

Molecular Brain Research ◽

10.1016/s0169-328x(98)00290-3 ◽

1999 ◽

Vol 63 (2) ◽

pp. 268-275 ◽

Cited By ~ 2

Author(s):

Paolo Follesa ◽

Alessandra Mallei ◽

Stefania Floris ◽

Maria Cristina Mostallino ◽

Enrico Sanna ◽

...

Keyword(s):

Animal Model ◽

Gabaa Receptor ◽

Wilson’S Disease ◽

Wilson's Disease ◽

Receptor Subunit ◽

Long Evans ◽

The Brain

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Protection from spontaneous hepatocellular damage by N-benzyl-d-glucamine dithiocarbamate in Long–Evans Cinnamon rats, an animal model of Wilson's disease

Toxicology and Applied Pharmacology ◽

10.1016/j.taap.2004.06.005 ◽

2005 ◽

Vol 202 (1) ◽

pp. 59-67 ◽

Cited By ~ 5

Author(s):

Hideaki Shimada ◽

Makiko Takahashi ◽

Akinori Shimada ◽

Tadashi Okawara ◽

Akira Yasutake ◽

...

Keyword(s):

Animal Model ◽

Wilson’S Disease ◽

Wilson's Disease ◽

Hepatocellular Damage ◽

Long Evans

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Microstructure changes in whiter matter relate to cognitive impairment in Wilson’s disease

Bioscience Reports ◽

10.1042/bsr20181651 ◽

2019 ◽

Vol 39 (3) ◽

Cited By ~ 1

Author(s):

Ting Dong ◽

Wen-ming Yang ◽

Ming-cai Wu ◽

Juan Zhang ◽

Peng Huang ◽

...

Keyword(s):

Cognitive Impairment ◽

Cognitive Function ◽

Wilson’S Disease ◽

Diffusion Tensor ◽

Genetic Disorder ◽

Brain Mri ◽

Wilson's Disease ◽

Copper Accumulation ◽

Lentiform Nucleus ◽

Impaired Cognitive Function

Abstract Purpose: Wilson’s disease (WD) is a genetic disorder of copper metabolism with pathological copper accumulation in the brain. The purpose of the present study was to evaluate the relationship between the damaged white matter and the impaired cognitive function in WD patients. Materials and methods: Thirty WD adolescents and thirty age- and sex-matched healthy controls (HC) were enrolled. All subjects had received brain MRI, including conventional and diffusion-tensor imaging (DTI) scans. The DTI parameter of fractional anisotropy (FA) was calculated by diffusion kurtosis estimator software. The t test was used to compare the differences between two groups. The correlation between cognitive function and whiter matter disorders were analyzed by linear regression. The results of FA parameter and MD parameter intergroup analysis were both corrected with False Discovery Rate (FDR) simulations by SPSS. Results: WD adolescents showed significantly lower scores of time-based prospective memory (TBPM) and verbal fluency test (VFT) compared with HC. We found significantly higher FA in the right thalamus, right lentiform nucleus, left thalamus, left lentiform nucleus, and brain stem in WD adolescents. Besides, WD adolescents exhibited significantly lower FA in right cerebellum and cingulum and left middle frontal lobe compared with controls (P<0.05). There were significantly negative correlations between FA in bilateral lentiform and thalamus and cognitive impairment in WD adolescents (P<0.05). Conclusion: The whiter matter of WD adolescents was impaired and mainly distributed in subcortical brain regions. The impaired cognitive function was affected by the damaged whiter matter. The present study may be helpful for recognition and understanding of WD.

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