Animal models of Wilson disease

Journal of Neurochemistry
Emily ReedOliver Bandmann

Abstract

Wilson disease (WD) is an autosomal recessive disorder of copper metabolism manifesting with hepatic, neurological and psychiatric symptoms. The limitations of the currently available therapy for WD (particularly in the management of neuropsychiatric disease), together with our limited understanding of key aspects of this illness (e.g. neurological vs. hepatic presentation) justify the ongoing need to study WD in suitable animal models. Four animal models of WD have been established: the Long-Evans Cinnamon rat, the toxic-milk mouse, the Atp7b knockout mouse and the Labrador retriever. The existing models of WD all show good similarity to human hepatic WD and have been helpful in developing an improved understanding of the human disease. As mammals, the mouse, rat and canine models also benefit from high homology to the human genome. However, important differences exist between these mammalian models and human disease, particularly the absence of a convincing neurological phenotype. This review will first provide an overview of our current knowledge of the orthologous genes encoding ATP7B and the closely related ATP7A protein in C. elegans, Drosophila and zebrafish (Danio rerio) and then summarise key characteristics of rodent ...Continue Reading

References

Jan 1, 1992·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Y TogashiH Kobayashi
Aug 1, 1990·Biological Psychiatry·T R Dening, G E Berrios
Nov 1, 1987·The Journal of Heredity·M C YoshidaM Mori
May 1, 1983·The Journal of Heredity·H Rauch
Apr 6, 1995·Biochemical and Biophysical Research Communications·Y MurataM Kodama
Apr 1, 1994·The Tohoku Journal of Experimental Medicine·S AbeK Suzuki
Jan 1, 1994·Journal of Comparative Pathology·J M Howell, J F Mercer
Dec 1, 1993·The Biochemical Journal·J Koropatnick, M G Cherian
Nov 1, 1995·Pharmacology, Biochemistry, and Behavior·R L HawkinsK Torii
Oct 1, 1996·Human Molecular Genetics·M B TheophilosJ F Mercer
Feb 24, 1997·Biochemical and Biophysical Research Communications·M NakamuraT Ohyama
May 30, 1998·Proceedings of the National Academy of Sciences of the United States of America·S Lutsenko, M J Cooper
Sep 28, 2000·Biochemical and Biophysical Research Communications·M HayashiT Okui
Mar 10, 2001·Biochemical and Biophysical Research Communications·I VoskoboinikJ Camakaris
Mar 29, 2001·Molecular Therapy : the Journal of the American Society of Gene Therapy·A N IraniS Gupta

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Citations

Dec 19, 2018·Journal of Veterinary Internal Medicine·Hajime AsadaKoichi Ohno
Nov 7, 2019·Journal of Feline Medicine and Surgery·Hajime AsadaKoichi Ohno
Sep 11, 2019·The Journal of Nutrition·Xiaoyu WangJames F Collins
Jul 15, 2020·Journal of Molecular Neuroscience : MN·Jayantee KalitaSunil Kumar
Sep 2, 2020·Food and Chemical Toxicology : an International Journal Published for the British Industrial Biological Research Association·Jayant Patwa, S J S Flora
Mar 7, 2021·Animals : an Open Access Journal From MDPI·Ronald Jan Corbee, Louis C Penning
Jul 21, 2020·Journal of Hepatology·Federico PietrocolaGuido Kroemer
Jul 10, 2021·Reviews in Endocrine & Metabolic Disorders·Rubén EscribáÁngel Raya
Aug 4, 2021·Journal of Neurology, Neurosurgery, and Psychiatry·Samuel ShribmanThomas T Warner
Nov 7, 2021·Mammalian Genome : Official Journal of the International Mammalian Genome Society·M L KaldunskiA E Kwitek

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