scholarly journals Elevated Copper Remodels Hepatic RNA Processing Machinery in the Mouse Model of Wilson's Disease

2011 ◽  
Vol 406 (1) ◽  
pp. 44-58 ◽  
Author(s):  
Jason L. Burkhead ◽  
Martina Ralle ◽  
Phillip Wilmarth ◽  
Larry David ◽  
Svetlana Lutsenko
Keyword(s):  
Mouse Model ◽  
Rna Processing ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Processing Machinery

scholarly journals A systems approach implicates nuclear receptor targeting in the Atp7b−/− mouse model of Wilson's disease

Metallomics ◽  
2012 ◽  
Vol 4 (7) ◽  
pp. 660 ◽  
Author(s):  
Phillip A. Wilmarth ◽  
Kristopher K. Short ◽  
Oliver Fiehn ◽  
Svetlana Lutsenko ◽  
Larry L. David ◽  
...  
Keyword(s):  
Mouse Model ◽  
Nuclear Receptor ◽  
Wilson’S Disease ◽  
Systems Approach ◽  
Wilson's Disease ◽  
Receptor Targeting

Liver cell transplantation leads to repopulation and functional correction in a mouse model of Wilson's disease

2004 ◽  
Vol 19 (11) ◽  
pp. 1283-1290 ◽  
Author(s):  
KATRINA J ALLEN ◽  
DAPHNE MY CHEAH ◽  
PAUL FA WRIGHT ◽  
SOPHIE GAZEAS ◽  
NICOLE E PETTIGREW-BUCK ◽  
...  
Keyword(s):  
Mouse Model ◽  
Cell Transplantation ◽  
Liver Cell ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Functional Correction ◽  
Liver Cell Transplantation

Fragmentation of mitochondrial cardiolipin by copper ions in the Atp7b−/− mouse model of Wilson's disease

2011 ◽  
Vol 164 (5) ◽  
pp. 393-400 ◽  
Author(s):  
Irina L. Yurkova ◽  
Juergen Arnhold ◽  
Guenther Fitzl ◽  
Dominik Huster
Keyword(s):  
Mouse Model ◽  
Wilson’S Disease ◽  
Copper Ions ◽  
Wilson's Disease

scholarly journals GanDouLing combined with Penicillamine improves cerebrovascular injury via PERK/eIF2α/CHOP endoplasmic reticulum stress pathway in the mouse model of Wilson’s disease

2018 ◽  
Vol 38 (5) ◽  
Author(s):  
Yonghua Chen ◽  
Bo Zhang ◽  
Shijian Cao ◽  
Wei Huang ◽  
Ni Liu ◽  
...  
Keyword(s):  
Endoplasmic Reticulum ◽  
Mouse Model ◽  
Er Stress ◽  
Vascular Injury ◽  
Caspase 3 ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Cerebrovascular Injury ◽  
Caspase 12 ◽  
Stress Pathway

We aim to investigate the function and mechanism of GanDouLing combinated with Penicillamine on cerebrovascular injury in Wilson’s disease (WD). ELISA was performed to analyze the expression of vascular injury factors. Pathological changes of cerebral vessels were observed by HE stain. Immunohistochemistry assays were performed to analyze the expression of ICAM-1, VCAM-1, and GRP78. Western blotting was measured to analyze the expression of caspase-3, caspase-12, PERK, eIF2α, and CHOP. Apoptosis was detected with TUNEL assay. The expression of vascular injury factors and ICAM-1, VCAM-1 was significantly increased by WD and markedly decreased in GanDouLing-Penicillamine group. The expression of caspase-3, caspase-12, PERK, eIF2α, and CHOP were obviously expressed in Wilson group, GanDouLing-Penicillamine suppressed apoptosis and endoplasmic reticulum (ER) stress. Our findings suggested that GanDouLing-Penicillamine improved cerebrovascular injury through PERK/eIF2α/CHOP ER stress pathway in the mouse model of WD.

scholarly journals The Potential of Bone Marrow Stem Cells to Correct Liver Dysfunction in a Mouse Model of Wilson's Disease

2004 ◽  
Vol 13 (7-8) ◽  
pp. 765-774 ◽  
Author(s):  
Katrina J. Allen ◽  
Daphne M. Y. Cheah ◽  
Xiao Ling Lee ◽  
Nicole E. Pettigrew-Buck ◽  
Jim Vadolas ◽  
...  
Keyword(s):  
Stem Cells ◽  
Bone Marrow ◽  
Mouse Model ◽  
Liver Dysfunction ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Bone Marrow Stem Cells

scholarly journals Urinary Copper Elevation in a Mouse Model of Wilson's Disease Is a Regulated Process to Specifically Decrease the Hepatic Copper Load

PLoS ONE ◽  
2012 ◽  
Vol 7 (6) ◽  
pp. e38327 ◽  
Author(s):  
Lawrence W. Gray ◽  
Fangyu Peng ◽  
Shannon A. Molloy ◽  
Venkata S. Pendyala ◽  
Abigael Muchenditsi ◽  
...  
Keyword(s):  
Mouse Model ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Hepatic Copper ◽  
Urinary Copper

scholarly journals Neuroinflammatory and behavioural changes in the Atp7B mutant mouse model of Wilson’s disease

2011 ◽  
Vol 118 (1) ◽  
pp. 105-112 ◽  
Author(s):  
Dick Terwel ◽  
Yi-Na Löschmann ◽  
Hartmut H.-J. Schmidt ◽  
Hans R. Schöler ◽  
Tobias Cantz ◽  
...  
Keyword(s):  
Mouse Model ◽  
Wilson’S Disease ◽  
Mutant Mouse ◽  
Wilson's Disease ◽  
Behavioural Changes
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