A High-Calorie Diet Aggravates Mitochondrial Dysfunction and Triggers Severe Liver Damage in Wilson Disease Rats

Ferenci, 2014, Phenotype-genotype correlations in patients with Wilson's disease, Ann N Y Acad Sci, 1315, 1, 10.1111/nyas.12340 Ferenci, 2012, EASL clinical practice guidelines: Wilson's disease, J Hepatol, 56, 671, 10.1016/j.jhep.2011.11.007 Ahmed, 2005, A new strain of rat for functional analysis of PINA, Brain Res Mol Brain Res, 137, 63, 10.1016/j.molbrainres.2005.02.025 Zischka, 2011, Liver mitochondrial membrane crosslinking and destruction in a rat model of Wilson disease, J Clin Invest, 121, 1508, 10.1172/JCI45401 Lichtmannegger, 2016, Methanobactin reverses acute liver failure in a rat model of Wilson disease, J Clin Invest, 126, 2721, 10.1172/JCI85226 Choi, 2006, Spectral, kinetic, and thermodynamic properties of Cu(I) and Cu(II) binding by methanobactin from Methylosinus trichosporium OB3b, Biochemistry, 45, 1442, 10.1021/bi051815t El Ghazouani, 2011, Copper-binding properties and structures of methanobactins from Methylosinus trichosporium OB3b, Inorg Chem, 50, 1378, 10.1021/ic101965j Sternlieb, 1968, Mitochondrial and fatty changes in hepatocytes of patients with Wilson's disease, Gastroenterology, 55, 354, 10.1016/S0016-5085(19)34045-4 Stattermayer, 2015, Hepatic steatosis in Wilson disease--role of copper and PNPLA3 mutations, J Hepatol, 63, 156, 10.1016/j.jhep.2015.01.034 Vernon, 2011, Systematic review: the epidemiology and natural history of non-alcoholic fatty liver disease and non-alcoholic steatohepatitis in adults, Aliment Pharmacol Ther, 34, 274, 10.1111/j.1365-2036.2011.04724.x Cortez-Pinto, 1999, Alterations in liver ATP homeostasis in human nonalcoholic steatohepatitis: a pilot study, JAMA, 282, 1659, 10.1001/jama.282.17.1659 Caldwell, 1999, Mitochondrial abnormalities in non-alcoholic steatohepatitis, J Hepatol, 31, 430, 10.1016/S0168-8278(99)80033-6 Einer, 2018, Mitochondrial adaptation in steatotic mice, Mitochondrion, 40, 1, 10.1016/j.mito.2017.08.015 Einer, 2017, Data on chow, liver tissue and mitochondrial fatty acid compositions as well as mitochondrial proteome changes after feeding mice a western diet for 6-24 weeks, Data Brief, 15, 163, 10.1016/j.dib.2017.09.019 Pfeiffer, 2011, Wilson's disease, Handb Clin Neurol, 100, 681, 10.1016/B978-0-444-52014-2.00049-5 Brewer, 1993, Does a vegetarian diet control Wilson's disease?, J Am Coll Nutr, 12, 527, 10.1080/07315724.1993.10718347 Kegley, 2010, Fulminant Wilson's disease requiring liver transplantation in one monozygotic twin despite identical genetic mutation, Am J Transplant, 10, 1325, 10.1111/j.1600-6143.2010.03071.x Kieffer, 2017, Wilson disease: at the crossroads between genetics and epigenetics-a review of the evidence, Liver Res, 1, 121, 10.1016/j.livres.2017.08.003 Yamazaki, 1993, Effects of dietary vitamin E on clinical course and plasma glutamic oxaloacetic transaminase and glutamic pyruvic transaminase activities in hereditary hepatitis of LEC rats, Lab Anim Sci, 43, 61 Kitamura, 2005, Effects of N-acetylcysteine, quercetin, and phytic acid on spontaneous hepatic and renal lesions in LEC rats, Toxicol Pathol, 33, 584, 10.1080/01926230500246675 Levy, 2007, Abnormal hepatobiliary and circulating lipid metabolism in the Long-Evans Cinnamon rat model of Wilson's disease, Life Sci, 80, 1472, 10.1016/j.lfs.2007.01.017 Huster, 2007, High copper selectively alters lipid metabolism and cell cycle machinery in the mouse model of Wilson disease, J Biol Chem, 282, 8343, 10.1074/jbc.M607496200 Pierson, 2018, The function of ATPase copper transporter ATP7B in intestine, Gastroenterology, 154, 168, 10.1053/j.gastro.2017.09.019 Tetri, 2008, Severe NAFLD with hepatic necroinflammatory changes in mice fed trans fats and a high-fructose corn syrup equivalent, Am J Physiol Gastrointest Liver Physiol, 295, G987, 10.1152/ajpgi.90272.2008 Kleiner, 2005, Design and validation of a histological scoring system for nonalcoholic fatty liver disease, Hepatology, 41, 1313, 10.1002/hep.20701 Tao, 2003, Hepatic copper metabolism: insights from genetic disease, Hepatology, 37, 1241, 10.1053/jhep.2003.50281 Nobili, 2013, Levels of serum ceruloplasmin associate with pediatric nonalcoholic fatty liver disease, J Pediatr Gastroenterol Nutr, 56, 370, 10.1097/MPG.0b013e31827aced4 Aigner, 2010, A role for low hepatic copper concentrations in nonalcoholic fatty liver disease, Am J Gastroenterol, 105, 1978, 10.1038/ajg.2010.170 Sternlieb, 1979, Electron microscopy of mitochondria and peroxisomes of human hepatocytes, Prog Liver Dis, 6, 81 Roberts, 2008, Mitochondrial structure and function in the untreated Jackson toxic milk (tx-j) mouse, a model for Wilson disease, Mol Genet Metab, 93, 54, 10.1016/j.ymgme.2007.08.127 Serviddio, 2008, Alterations of hepatic ATP homeostasis and respiratory chain during development of non-alcoholic steatohepatitis in a rodent model, Eur J Clin Invest, 38, 245, 10.1111/j.1365-2362.2008.01936.x McGarry, 1969, Ketogenesis and cholesterol synthesis in normal and neoplastic tissues of the rat, J Biol Chem, 244, 4251, 10.1016/S0021-9258(17)36409-8 Koliaki, 2015, Adaptation of hepatic mitochondrial function in humans with non-alcoholic fatty liver is lost in steatohepatitis, Cell Metab, 21, 739, 10.1016/j.cmet.2015.04.004 Bandmann, 2015, Wilson's disease and other neurological copper disorders, Lancet Neurol, 14, 103, 10.1016/S1474-4422(14)70190-5 Czlonkowska, 2009, Monozygotic female twins discordant for phenotype of Wilson's disease, Mov Disord, 24, 1066, 10.1002/mds.22474 Medici, 2017, Genetic and environmental modifiers of Wilson disease, Handb Clin Neurol, 142, 35, 10.1016/B978-0-444-63625-6.00004-5 Schiefermeier, 2000, The impact of apolipoprotein E genotypes on age at onset of symptoms and phenotypic expression in Wilson's disease, Brain, 123, 585, 10.1093/brain/123.3.585 Litwin, 2012, Apolipoprotein E gene (APOE) genotype in Wilson's disease: impact on clinical presentation, Parkinsonism Relat Disord, 18, 367, 10.1016/j.parkreldis.2011.12.005 Rodo, 2000, The level of serum lipids, vitamin E and low density lipoprotein oxidation in Wilson's disease patients, Eur J Neurol, 7, 491, 10.1046/j.1468-1331.2000.t01-1-00115.x Seessle, 2011, Alterations of lipid metabolism in Wilson disease, Lipids Health Dis, 10, 83, 10.1186/1476-511X-10-83 Ralle, 2010, Wilson disease at a single cell level: intracellular copper trafficking activates compartment-specific responses in hepatocytes, J Biol Chem, 285, 30875, 10.1074/jbc.M110.114447 Newman, 2012, Mitochondrial protein acylation and intermediary metabolism: regulation by sirtuins and implications for metabolic disease, J Biol Chem, 287, 42436, 10.1074/jbc.R112.404863 Menzies, 2016, Protein acetylation in metabolism - metabolites and cofactors, Nat Rev Endocrinol, 12, 43, 10.1038/nrendo.2015.181 Hirschey, 2011, SIRT3 deficiency and mitochondrial protein hyperacetylation accelerate the development of the metabolic syndrome, Mol Cell, 44, 177, 10.1016/j.molcel.2011.07.019 Arguello, 2015, Recent insights on the role of cholesterol in non-alcoholic fatty liver disease, Biochim Biophys Acta, 1852, 1765, 10.1016/j.bbadis.2015.05.015 Schulz, 2013, Progressive stages of mitochondrial destruction caused by cell toxic bile salts, Biochim Biophys Acta, 1828, 2121, 10.1016/j.bbamem.2013.05.007 Hohenester, 2010, Phosphatidylinositol-3-kinase p110γ contributes to bile salt-induced apoptosis in primary rat hepatocytes and human hepatoma cells, J Hepatol, 53, 918, 10.1016/j.jhep.2010.05.015 Weiss J. Haus- und versuchstierpflege: 80 Tabellen: Enke, Stuttgart, Germany 2003. Schosinsky, 1974, Measurement of ceruloplasmin from its oxidase activity in serum by use of o-dianisidine dihydrochloride, Clin Chem, 20, 1556, 10.1093/clinchem/20.12.1556 Erel, 1998, Automated measurement of serum ferroxidase activity, Clin Chem, 44, 2313, 10.1093/clinchem/44.11.2313 Ishak, 1995, Histological grading and staging of chronic hepatitis, J Hepatol, 22, 696, 10.1016/0168-8278(95)80226-6 Zischka, 2008, Electrophoretic analysis of the mitochondrial outer membrane rupture induced by permeability transition, Anal Chem, 80, 5051, 10.1021/ac800173r Schmitt, 2013, A semi-automated method for isolating functionally intact mitochondria from cultured cells and tissue biopsies, Anal Biochem, 443, 66, 10.1016/j.ab.2013.08.007 Schulz, 2015, A protocol for the parallel isolation of intact mitochondria from rat liver, kidney, heart, and brain, Methods Mol Biol, 1295, 75, 10.1007/978-1-4939-2550-6_7 Muller, 2008, High rates of superoxide production in skeletal-muscle mitochondria respiring on both complex I- and complex II-linked substrates, Biochem J, 409, 491, 10.1042/BJ20071162 Schmitt, 2014, Why to compare absolute numbers of mitochondria, Mitochondrion, 19, 113, 10.1016/j.mito.2014.06.005 Saggerson, 1986, Carnitine palmitoyltransferase in liver and five extrahepatic tissues in the rat. Inhibition by DL-2-bromopalmitoyl-CoA and effect of hypothyroidism, Biochem J, 236, 137, 10.1042/bj2360137 Williams, 1998, Automated analysis of mitochondrial enzymes in cultured skin fibroblasts, Anal Biochem, 259, 176, 10.1006/abio.1998.2624 Hackenbrock, 1968, Ultrastructural bases for metabolically linked mechanical activity in mitochondria. II. Electron transport-linked ultrastructural transformations in mitochondria, J Cell Biol, 37, 345, 10.1083/jcb.37.2.345 von Toerne, 2013, Apoe, Mbl2, and Psp plasma protein levels correlate with diabetic phenotype in NZO mice--an optimized rapid workflow for SRM-based quantification, J Proteome Res, 12, 1331, 10.1021/pr3009836 Obermann, 2017, Proteome-wide identification of glycosylation-dependent interactors of galectin-1 and galectin-3 on mesenchymal retinal pigment epithelial (RPE) cells, Mol Cell Proteomics, 16, 1528, 10.1074/mcp.M116.066381 Hachmoller, 2016, Element bioimaging of liver needle biopsy specimens from patients with Wilson's disease by laser ablation-inductively coupled plasma-mass spectrometry, J Trace Elem Med Biol, 35, 97, 10.1016/j.jtemb.2016.02.001 Bandow, 2011, Isolation of methanobactin from the spent media of methane-oxidizing bacteria, Methods Enzymol, 495, 259, 10.1016/B978-0-12-386905-0.00017-6 Bradford, 1976, A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding, Anal Biochem, 72, 248, 10.1016/0003-2697(76)90527-3