T. Tao and J. Gitlin, Hepatic copper metabolism: Insights from genetic disease, Hepatology, vol.38, issue.6, pp.1241-1247, 2003.
DOI : 10.1053/jhep.2003.50281

A. Ala, A. Walker, and K. Ashkan, Wilson's disease, The Lancet, vol.369, issue.9559, pp.397-408, 2007.
DOI : 10.1016/S0140-6736(07)60196-2

A. Coffey, M. Durkie, and S. Hague, 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

K. Dziezyc, T. Litwin, and G. Chabik, Families with Wilson's disease in subsequent generations: Clinical and genetic analysis, Movement Disorders, vol.89, issue.14
DOI : 10.1002/mds.26057

M. Schilsky and A. Tavill, Wilson Disease, Disease of the Liver Lippicott Williams & Wilkins: Philadelphia, pp.1169-1186, 2003.
DOI : 10.1002/9781119950509.ch29

N. Holtzman and B. Gaumnitz, Studies on the rate of release and turnover of ceruloplasmin and apoceruloplasmin in rat plasma

P. Ferenci, Wilson???s Disease, Clinical Gastroenterology and Hepatology, vol.3, issue.8, pp.726-733, 2005.
DOI : 10.1016/S1542-3565(05)00484-2

P. Steindl, P. Ferenci, and H. Dienes, 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

E. Cauza, T. Maier-dobersberger, and P. Ferenci, 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

S. Das and K. Ray, Wilson's disease: an update, Nature Clinical Practice Neurology, vol.40, issue.9, pp.482-493, 2006.
DOI : 10.1038/ncpneuro0291

E. Roberts and D. Cox, 3 Wilson disease, Bailli??re's Clinical Gastroenterology, vol.12, issue.2, pp.237-256, 1998.
DOI : 10.1016/S0950-3528(98)90133-6

E. Roberts and M. Schilsky, Diagnosis and treatment of Wilson disease: An update, Hepatology, vol.11, issue.6, pp.2089-2111, 2008.
DOI : 10.1002/hep.22261

E. Balkhi, S. Trocello, J. Poupon, and J. , 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

J. Trocello, E. Balkhi, S. Woimant, and F. , 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

N. Bader, Sample preparation for flame atomic absorption spectroscopy: an overview, Rasayan J Chem, vol.4, pp.49-55, 2011.

S. Goldfischer, H. Popper, and I. Sternlieb, The significance of variations in the distribution of copper in liver disease, Am J Pathol, vol.99, pp.715-730, 1980.

M. De-jonge, C. Holzner, and S. Baines, 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

S. Majumdar, J. Peralta-videa, and H. Castillo-michel, 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

H. Kinoshita, Y. Hori, and T. Fukumoto, 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

Y. Liu, L. Li, and Y. Gao, Distribution of metalloproteins in hepatocellular carcinoma and surrounding tissues, Hepatogastroenterology, vol.54, pp.2291-2296, 2007.

W. Osterode, G. Falkenberg, and R. Hoftberger, 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

L. Naour, F. Sandt, C. Peng, and C. , 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

M. Ralle, N. Blackburn, and S. Lutsenko, Using XAS and SXRF to Study Copper in Wilson Disease at the Molecular and Tissue Level, AIP Conference Proceedings
DOI : 10.1063/1.2644515

M. Belkin, R. Zeimer, and T. Chajek, Non-invasive quantitation on corneal copper in hepatolenticular degeneration, Lancet, vol.21, pp.391-392, 1976.

R. Deslattes, E. Kessler, and P. Indelicato, X-ray Transition Energies (version 1.2). [Online] Available

V. Sol-e, E. Papillon, and M. Cotte, 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

W. Vonk, C. Wijmenga, and B. Van-de-sluis, Relevance of animal models for understanding mammalian copper homeostasis, Am J Clin Nutr, vol.88, pp.840-845, 2008.

K. Suzuki, S. Kanno, and S. Misawa, 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

R. Masuda, M. Yoshida, and M. Sasaki, 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

M. Hayashi, S. Fuse, and D. Endoh, 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

S. Goldfischer and I. Sternlieb, 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.

S. Material, . On, and . Internet, The following supplementary material may be found in the online version of this article: Supplementary Table 1