Hepatic copper metabolism: Insights from genetic disease, Hepatology, vol.38, issue.6, pp.1241-1247, 2003. ,
DOI : 10.1053/jhep.2003.50281
Wilson's disease, The Lancet, vol.369, issue.9559, pp.397-408, 2007. ,
DOI : 10.1016/S0140-6736(07)60196-2
A genetic study of Wilson???s disease in the United Kingdom, Brain, vol.136, issue.5, pp.1476-1487, 2013. ,
DOI : 10.1093/brain/awt035
Families with Wilson's disease in subsequent generations: Clinical and genetic analysis, Movement Disorders, vol.89, issue.14 ,
DOI : 10.1002/mds.26057
Wilson Disease, Disease of the Liver Lippicott Williams & Wilkins: Philadelphia, pp.1169-1186, 2003. ,
DOI : 10.1002/9781119950509.ch29
Studies on the rate of release and turnover of ceruloplasmin and apoceruloplasmin in rat plasma ,
Wilson???s Disease, Clinical Gastroenterology and Hepatology, vol.3, issue.8, pp.726-733, 2005. ,
DOI : 10.1016/S1542-3565(05)00484-2
Wilson's disease in patients presenting with liver disease: A diagnostic challenge, Gastroenterology, vol.113, issue.1, pp.212-218, 1997. ,
DOI : 10.1016/S0016-5085(97)70097-0
Screening for Wilson's disease in patients with liver diseases by serum ceruloplasmin, Journal of Hepatology, vol.27, issue.2, pp.358-362, 1997. ,
DOI : 10.1016/S0168-8278(97)80182-1
Wilson's disease: an update, Nature Clinical Practice Neurology, vol.40, issue.9, pp.482-493, 2006. ,
DOI : 10.1038/ncpneuro0291
3 Wilson disease, Bailli??re's Clinical Gastroenterology, vol.12, issue.2, pp.237-256, 1998. ,
DOI : 10.1016/S0950-3528(98)90133-6
Diagnosis and treatment of Wilson disease: An update, Hepatology, vol.11, issue.6, pp.2089-2111, 2008. ,
DOI : 10.1002/hep.22261
Relative exchangeable copper: A new highly sensitive and highly specific biomarker for Wilson's disease diagnosis, Clinica Chimica Acta, vol.412, issue.23-24 ,
DOI : 10.1016/j.cca.2011.08.019
Relative exchangeable copper: A promising tool for family screening in Wilson disease, Movement Disorders, vol.50, issue.4, pp.558-562, 2014. ,
DOI : 10.1002/mds.25763
Sample preparation for flame atomic absorption spectroscopy: an overview, Rasayan J Chem, vol.4, pp.49-55, 2011. ,
The significance of variations in the distribution of copper in liver disease, Am J Pathol, vol.99, pp.715-730, 1980. ,
Quantitative 3D elemental microtomography of Cyclotella meneghiniana at 400-nm resolution, Proceedings of the National Academy of Sciences, vol.107, issue.36, pp.15676-15680, 2010. ,
DOI : 10.1073/pnas.1001469107
Applications of synchrotron ??-XRF to study the distribution of biologically important elements in different environmental matrices: A review, Analytica Chimica Acta, vol.755, pp.1-16, 2012. ,
DOI : 10.1016/j.aca.2012.09.050
Novel assessment of hepatic iron distribution by synchrotron radiation X-ray fluorescence microscopy, Medical Molecular Morphology, vol.21, issue.suppl1, pp.19-25, 2010. ,
DOI : 10.1007/s00795-009-0474-7
Distribution of metalloproteins in hepatocellular carcinoma and surrounding tissues, Hepatogastroenterology, vol.54, pp.2291-2296, 2007. ,
Iron, copper, zinc and bromine mapping in cirrhotic liver slices from patients with hemochromatosis studied by microscopic synchrotron radiation X-ray fluorescence analysis in continuous scanning mode, Spectrochimica Acta Part B: Atomic Spectroscopy, vol.62, issue.6-7, pp.682-688, 2007. ,
DOI : 10.1016/j.sab.2007.03.031
In Situ Chemical Composition Analysis of Cirrhosis by Combining Synchrotron Fourier Transform Infrared and Synchrotron X-ray Fluorescence Microspectroscopies on the Same Tissue Section, Analytical Chemistry, vol.84, issue.23, pp.10260-10266, 2012. ,
DOI : 10.1021/ac302072t
Using XAS and SXRF to Study Copper in Wilson Disease at the Molecular and Tissue Level, AIP Conference Proceedings ,
DOI : 10.1063/1.2644515
Non-invasive quantitation on corneal copper in hepatolenticular degeneration, Lancet, vol.21, pp.391-392, 1976. ,
X-ray Transition Energies (version 1.2). [Online] Available ,
A multiplatform code for the analysis of energy-dispersive X-ray fluorescence spectra, Spectrochimica Acta Part B: Atomic Spectroscopy, vol.62, issue.1, pp.63-68, 2007. ,
DOI : 10.1016/j.sab.2006.12.002
Relevance of animal models for understanding mammalian copper homeostasis, Am J Clin Nutr, vol.88, pp.840-845, 2008. ,
Copper metabolism leading to and following acute hepatitis in LEC rats, Toxicology, vol.97, issue.1-3, pp.81-92, 1995. ,
DOI : 10.1016/0300-483X(94)02927-M
Hereditary hepatitis of LEC rats is controlled by a single autosomal recessive gene, Laboratory Animals, vol.22, issue.2, pp.166-169, 1988. ,
DOI : 10.1258/002367788780864402
Accumulation of Copper Induces DNA Strand Breaks in Brain Cells of Long-Evans Cinnamon (LEC) Rats, An Animal Model for Human Wilson Disease, Experimental Animals, vol.55, issue.5, pp.419-426, 2006. ,
DOI : 10.1538/expanim.55.419
Changes in the distribution of hepatic copper in relation to the progression of Wilson's disease, Am J Pathol, vol.53, pp.883-901, 1968. ,
The following supplementary material may be found in the online version of this article: Supplementary Table 1 ,