inbred lec rats

Summary

Summary: A cinnamon-colored strain of Long-Evans rats which carries a mutation causing fulminant hepatitis and jaundice, with an associated gross accumulation of copper in the liver. This strain is a model for Wilson's Disease (see HEPATOLENTICULAR DEGENERATION).

Top Publications

  1. Okui T, Endoh D, Kon Y, Hayashi M. Deficiency in nuclear accumulation of G22p1 and Xrcc5 proteins in hyper-radiosensitive Long-Evans Cinnamon (LEC) rat cells after X irradiation. Radiat Res. 2002;157:553-61 pubmed
    ..Since the nucleotide sequences of the G22p1 and Xrcc5 genes of the LEC rats coincided with those of the control rats, the deficiency in nuclear accumulation may not be caused by mutations of the G22p1 and Xrcc5 proteins. ..
  2. Klein D, Lichtmannegger J, Finckh M, Summer K. Gene expression in the liver of Long-Evans cinnamon rats during the development of hepatitis. Arch Toxicol. 2003;77:568-75 pubmed
    ..During the progression of the liver disease, in particular genes related to oxidative stress, DNA damage, apoptosis and inflammation with acute-phase reaction were upregulated. ..
  3. Tsubota A, Matsumoto K, Mogushi K, Nariai K, Namiki Y, Hoshina S, et al. IQGAP1 and vimentin are key regulator genes in naturally occurring hepatotumorigenesis induced by oxidative stress. Carcinogenesis. 2010;31:504-11 pubmed publisher
    ..These findings enhance our understanding of the multistep hepatotumorigenesis and identification of target molecules for novel treatments. ..
  4. Tsuchigauchi T, Takahashi T, Ohnishi T, Ogawa H, Bando Y, Uehara H, et al. Low susceptibility to N-ethyl-N-nitrosourea-induced transplacental carcinogenesis in Long-Evans Cinnamon (LEC) rats. J Med Invest. 2009;56:93-8 pubmed
    ..Present results suggest that high levels of MT and/or MGMT contribute to the resistance to nitrosamine-induced carcinogenesis in LEC rats...
  5. Chiba M, Itagaki S, Kobayashi M, Hirano T, Iseki K. Characterization of hepatobiliary organic anion transporters in Long-Evans Cinnamon rats. Drug Metab Pharmacokinet. 2007;22:387-90 pubmed
    ..However, no significant difference of the expressions of Mrp2 and Bsep, organic anion transporters located on canalicular membrane, were found between Wistar rats and LEC rats. ..
  6. Malhi H, Irani A, Volenberg I, Schilsky M, Gupta S. Early cell transplantation in LEC rats modeling Wilson's disease eliminates hepatic copper with reversal of liver disease. Gastroenterology. 2002;122:438-47 pubmed
    ..Cell transplantation eventually restored copper homeostasis and reversed liver disease without hepatic preconditioning in LEC rats. ..
  7. Kitamura Y, Nishikawa A, Nakamura H, Furukawa F, Imazawa T, Umemura T, et al. Effects of N-acetylcysteine, quercetin, and phytic acid on spontaneous hepatic and renal lesions in LEC rats. Toxicol Pathol. 2005;33:584-92 pubmed
    ..In addition, it was suggested that the different effects of the anti-oxidants on lesion development in LEC rats might be related to different mechanisms of action with regard to oxidative stress. ..
  8. Nakayama K, Takasawa A, Terai I, Okui T, Ohyama T, Tamura M. Spontaneous porphyria of the Long-evans cinnamon rat: an animal model of Wilson's disease. Arch Biochem Biophys. 2000;375:240-50 pubmed
    ..This was independent of sex and age. The present work stresses the existence of an abnormal heme metabolism in the LEC rat, and thus, the necessity to study the heme metabolism in human Wilson's disease is strongly suggested. ..
  9. Malhi H, Bhargava K, Afriyie M, Volenberg I, Schilsky M, Palestro C, et al. 99mTc-mebrofenin scintigraphy for evaluating liver disease in a rat model of Wilson's disease. J Nucl Med. 2002;43:246-52 pubmed

More Information

Publications88

  1. Xu H, Sakakibara S, Morifuji M, Salamatulla Q, Aoyama Y. Excess dietary histidine decreases the liver copper level and serum alanine aminotransferase activity in Long-Evans Cinnamon rats. Br J Nutr. 2003;90:573-9 pubmed
    ..Thus, it may be of benefit in the prevention or treatment of liver injury in LEC rats and in patients with Wilson's disease. ..
  2. Marcangione C, Rompre P. Topographical Fos induction within the ventral midbrain and projection sites following self-stimulation of the posterior mesencephalon. Neuroscience. 2008;154:1227-41 pubmed publisher
    ..They provide additional support for the functional link between neurons that support PM and VTA self-stimulation, and offer topographical guidance to future attempts at their identification. ..
  3. Kim D, Ahn T, Kim J, Jeon G, Seo J, Jeon B, et al. Enhanced Mn-SOD immunoreactivity in the dopaminergic neurons of long-evans cinnamon rats. Neurochem Res. 2005;30:475-8 pubmed
    ..Our data suggest that LEC rats may contribute to the mechanistic study of neurological manifestations in nigro-striatal dopaminergic system of Wilson's disease. ..
  4. Medici V, Sturniolo G, Santon A, D Incà R, Bortolami M, Cardin R, et al. Efficacy of zinc supplementation in preventing acute hepatitis in Long-Evans Cinnamon rats. Liver Int. 2005;25:888-95 pubmed
    ..8-hydroxy-2'-deoxyguanosine amounts were significantly lower in untreated rats. Zn acetate prevents acute hepatitis, by increasing tissue MT concentrations, reducing Cu absorption and interfering with Fe metabolism. ..
  5. Kim J, Ko S, Kwon S, Kim H, Han M, Kim D, et al. Ferrous and ferric iron accumulates in the brain of aged Long-Evans Cinnamon rats, an animal model of Wilson's disease. Neurosci Lett. 2005;382:143-7 pubmed
    ..Further studies are required to elucidate the mechanisms of Cu and iron accumulations and of their effects. ..
  6. Suzuki K, Ogura Y. [Biological regulation of copper and selective removal of copper: therapy for Wilson disease and its molecular mechanism]. Yakugaku Zasshi. 2000;120:899-908 pubmed
  7. Itagaki S, Sugawara M, Kobayashi M, Miyazaki K, Hirano T, Iseki K. Comparison of the disposition behavior of organic anions in an animal model for Wilson's disease (Long-Evans Cinnamon rats) with that in normal Long-Evans Agouti rats. Drug Metab Pharmacokinet. 2004;19:150-4 pubmed
    ..It is important to elucidate the relationship between organic anion secretion and Wilson's disease. ..
  8. Lee S, Kim W, Ham B, Chen W, Bear M, Yoon B. Activity-dependent NR2B expression is mediated by MeCP2-dependent epigenetic regulation. Biochem Biophys Res Commun. 2008;377:930-4 pubmed publisher
    ..Together, these data indicate that DNA methylation as well as subsequent MeCP2 association mediates neuronal activity-dependent regulation of NR2B expressions. ..
  9. Choudhury S, Zhang R, Frenkel K, Kawamori T, Chung F, Roy R. Evidence of alterations in base excision repair of oxidative DNA damage during spontaneous hepatocarcinogenesis in Long Evans Cinnamon rats. Cancer Res. 2003;63:7704-7 pubmed
  10. MacMurray A, Moralejo D, Kwitek A, Rutledge E, Van Yserloo B, Gohlke P, et al. Lymphopenia in the BB rat model of type 1 diabetes is due to a mutation in a novel immune-associated nucleotide (Ian)-related gene. Genome Res. 2002;12:1029-39 pubmed
    ..The lymphopenia mutation may thus be useful not only to elucidate Type 1 diabetes, but also in the function of the Ian gene family as a whole...
  11. Lecain E, Yang T, Tran Ba Huy P. Steroidogenic enzyme expression in the rat cochlea. Acta Otolaryngol. 2003;123:187-91 pubmed
    ..Experiments aimed at demonstrating enzymatic activities within cochlea tissues are in progress. ..
  12. Jia G, Tohyama C, Sone H. DNA damage triggers imbalance of proliferation and apoptosis during development of preneoplastic foci in the liver of Long-Evans Cinnamon rats. Int J Oncol. 2002;21:755-61 pubmed
    ..These findings suggest that DNA damage, reflected in DNA strand breaks, plays a critical role in the development of hepatocellular preneoplastic foci, with an imbalance between high proliferation and relatively low apoptosis. ..
  13. Itagaki S, Shimamoto S, Hirano T, Iseki K, Sugawara M, Nishimura S, et al. Comparison of urinary excretion of phenolsulfonphthalein in an animal model for Wilson's disease (Long-Evans Cinnamon rats) with that in normal Wistar rats: involvement of primary active organic anion transporter. J Pharm Pharm Sci. 2004;7:227-34 pubmed
    ..The results suggest that PSP is transported by primary active organic anion transporter and that the activity level of this transporter is reduced in LEC rats. ..
  14. Shibata T, Nagayasu H, Kitajo H, Arisue M, Yamashita T, Hatakeyama D, et al. Inhibitory effects of fermented brown rice and rice bran on the development of acute hepatitis in Long-Evans Cinnamon rats. Oncol Rep. 2006;15:869-74 pubmed
    ..These results suggest that FBRA has preventive effects on the development of hepatitis in LEC rats and may play an important role in protecting the liver against the free radicals induced by copper accumulation in the liver. ..
  15. Pinato L, Allemandi W, Abe L, Frazao R, Cruz Rizzolo R, Cavalcante J, et al. A comparative study of cytoarchitecture and serotonergic afferents in the suprachiasmatic nucleus of primates (Cebus apella and Callithrix jacchus) and rats (Wistar and Long Evans strains). Brain Res. 2007;1149:101-10 pubmed
    ..These findings raise questions concerning the intrinsic organization of the nucleus, as well as regarding the functional relationship between serotonergic input and retinal afferents in this diurnal species. ..
  16. Knerlich Lukoschus F, Juraschek M, Blomer U, Lucius R, Mehdorn H, Held Feindt J. Force-dependent development of neuropathic central pain and time-related CCL2/CCR2 expression after graded spinal cord contusion injuries of the rat. J Neurotrauma. 2008;25:427-48 pubmed publisher
    ..These data demonstrate lesion grade dependence of below-level pain development and suggest chemokines as potential candidates for integrating inflammation and central neuropathic pain after SCI. ..
  17. Lentsch A, Crouch L, Jordan J, Czermak B, Yun E, Guo R, et al. Regulatory effects of interleukin-11 during acute lung inflammatory injury. J Leukoc Biol. 1999;66:151-7 pubmed
  18. Chang B, Nishikawa M, Nishiguchi S, Inoue M. L-carnitine inhibits hepatocarcinogenesis via protection of mitochondria. Int J Cancer. 2005;113:719-29 pubmed
    ..These facts suggest that mitochondrial injury plays an essential role in the development of hepatocarcinogenesis and that the clinical use of carnitine has excellent therapeutic potential in individuals with chronic hepatitis...
  19. Gyöngyi Z, Ember I, Kiss I, Varga C. Changes in expression of onco- and suppressor genes in peripheral leukocytes--as potential biomarkers of chemical carcinogenesis. Anticancer Res. 2001;21:3377-80 pubmed
    ..c-myc did not prove to be an applicable early biomarker due to the lack of or low level of its expression. However, remarkable of early elevations were detected in the expression signals of Ha-ras and p53...
  20. Shoda R, Mahalanabis D, Islam K, Wahed M, Albert M. Folic acid supplementation on red kidney bean-induced diarrhoea and enteric bacterial translocation into mesenteric lymph nodes in rats: a pilot study. Acta Paediatr. 2002;91:51-4 pubmed
    ..40 +/- 0.89 vs 1.42 +/- 1.31, p = 0.16) was documented. A significant positive correlation was also seen between the bacterial count in the jejunal mucosal scrapings and in the mesenteric lymph nodes...
  21. George G, Pickering I, Harris H, Gailer J, Klein D, Lichtmannegger J, et al. Tetrathiomolybdate causes formation of hepatic copper-molybdenum clusters in an animal model of Wilson's disease. J Am Chem Soc. 2003;125:1704-5 pubmed
    ..70 A. Each Cu is coordinated to 3-4 sulfurs at 2.28 A with approximately one Mo neighbor at 2.70 A. These results indicate the formation of a biologically novel molybdenum-copper-sulfur cluster...
  22. Hayashi M, Kuge T, Endoh D, Nakayama K, Arikawa J, Takazawa A, et al. No significant differences were observed in the amounts of DNA strand breaks produced by copper between male and female liver cells of long-evans cinnamon (LEC) strain rats. J Vet Med Sci. 2001;63:1109-13 pubmed
  23. Vu Q, McCarthy K, McCormack J, Schneeberger E. Lung dendritic cells are primed by inhaled particulate antigens, and retain MHC class II/antigenic peptide complexes in hilar lymph nodes for a prolonged period of time. Immunology. 2002;105:488-98 pubmed
    ..Antigen-loaded lung DC in hilar lymph nodes maintain their antigen presentation capacity for up to 2 weeks...
  24. Takeda K, Eguchi H, Soeda S, Shirahata A, Kawamura M. Fe(II)/Cu(I)-dependent P-type ATPase activity in the liver of Long-Evans cinnamon rats. Life Sci. 2005;76:2203-9 pubmed
    ..On the basis of these results, together with the similarity in optimal conditions required for full activity of the enzyme, we conclude that the Fe(II)-dependent ATPase (H-type) and WNDP may be identical...
  25. Meng Y, Miyoshi I, Hirabayashi M, Su M, Mototani Y, Okamura T, et al. Restoration of copper metabolism and rescue of hepatic abnormalities in LEC rats, an animal model of Wilson disease, by expression of human ATP7B gene. Biochim Biophys Acta. 2004;1690:208-19 pubmed
    ..This first successful transgenic rescue has important implications for the gene therapy of WD...
  26. Toshinai K, Mondal M, Shimbara T, Yamaguchi H, Date Y, Kangawa K, et al. Ghrelin stimulates growth hormone secretion and food intake in aged rats. Mech Ageing Dev. 2007;128:182-6 pubmed
    ..These findings suggest that the aged rats maintain the reactivity to administered exogenous ghrelin...
  27. Saito A, Nakayama K, Hara H. Mild zinc deficiency and dietary phytic acid accelerates the development of fulminant hepatitis in LEC rats. J Gastroenterol Hepatol. 2007;22:150-7 pubmed
    ..The aims of this study were to investigate the effects of mild zinc deficiency on the development of hepatitis and to determine the relationship between the absorption and hepatic levels of copper, zinc and iron...
  28. Gonzalez B, Niño Fong R, Gibson C, Fuentealba I, Cherian M. Zinc supplementation decreases hepatic copper accumulation in LEC rat: a model of Wilson's disease. Biol Trace Elem Res. 2005;105:117-34 pubmed
    ..Although the actual mechanism of protection is unknown, it could be explained by sequestration of dietary Cu by intestinal MT, induced by high dietary Zn content...
  29. Sajankila S, Manthena P, Adhikari S, Choudhury S, Izumi K, Roy R. Suppression of tumor suppressor Tsc2 and DNA repair glycosylase Nth1 during spontaneous liver tumorigenesis in Long-Evans Cinnamon rats. Mol Cell Biochem. 2010;338:233-9 pubmed publisher
    ..Increase in protein oxidation (carbonyl content) during the same time period (16-24 weeks) may have an effect on the promoter binding of regulatory proteins and consequent decrease in Nth1 and Tsc2 gene expressions during tumorigenesis...
  30. Tsuji A, Sugyo A, Ogiu T, Sagara M, Kimura T, Ishikawa A, et al. Fine mapping of radiation susceptibility and gene expression analysis of LEC congenic rat lines. Genomics. 2005;86:271-9 pubmed
    ..2-Mb region; however, expression analysis gave no clear indication as to which gene is responsible...
  31. Nair J, Strand S, Frank N, Knauft J, Wesch H, Galle P, et al. Apoptosis and age-dependant induction of nuclear and mitochondrial etheno-DNA adducts in Long-Evans Cinnamon (LEC) rats: enhanced DNA damage by dietary curcumin upon copper accumulation. Carcinogenesis. 2005;26:1307-15 pubmed
    ..LPO-induced adduct formation in nuclear and mitochondrial DNA appear as early lesions in LEC rat liver carcinogenesis and are discussed in relation to apoptotic events in the progression of malignant disease...
  32. Minami T, Kaneda S, Otsuka T, Jiao Z, Suzuki Y, Yamada T, et al. Role of Atp7b gene in spontaneous and N-diethylnitrosamine-induced carcinogenesis in a new congenic strain, WKAH.C-Atp7b rats. Jpn J Cancer Res. 2001;92:841-7 pubmed
    ..These data indicate that the susceptibility of LEC rats to liver and kidney carcinogenesis could be explained by Atp7b gene mutation and that the susceptibility to lung carcinogenesis is controlled by gene(s) other than Atp7b...
  33. Ueyama J, Wakusawa S, Tatsumi Y, Hattori A, Yano M, Hayashi H. Preliminary study of spontaneous hepatitis in Long-Evans Cinnamon rats: a blood exchange may improve fetal hepatitis. Nagoya J Med Sci. 2010;72:173-7 pubmed
    ..This would serve as a first step for establishing a treatment for fatal hepatitis in animals. A blood exchange may improve fulminant hepatitis of Wilson disease model rats...
  34. Watanabe S, Kitade Y, Masaki T, Nishioka M, Satoh K, Nishino H. Effects of lycopene and Sho-saiko-to on hepatocarcinogenesis in a rat model of spontaneous liver cancer. Nutr Cancer. 2001;39:96-101 pubmed
    ..There were no differences in serum alpha-fetoprotein levels or the cumulative survival rates between the groups. In conclusion, long-term administration of lycopene or TJ-9 did not reduce the risk of hepatocarcinogenesis in LEC rats...
  35. Márquez Quiñones A, Paris A, Roussel B, Perez Carreon J, Le Berre V, Francois J, et al. Proteasome activity deregulation in LEC rat hepatitis: following the insights of transcriptomic analysis. OMICS. 2007;11:367-84 pubmed
    ..These results bring new insights in the hepatitis and the hepatocarcinogenesis development...
  36. Yamamoto H, Watanabe T, Mizuno H, Endo K, Fukushige J, Hosokawa T, et al. The antioxidant effect of DL-alpha-lipoic acid on copper-induced acute hepatitis in Long-Evans Cinnamon (LEC) rats. Free Radic Res. 2001;34:69-80 pubmed
    ..Moreover, mitochondrial oxidative damage might be involved in the development of acute hepatitis in LEC rats...
  37. Kita Y, Masaki T, Funakoshi F, Yoshida S, Tanaka M, Kurokohchi K, et al. Expression of G1 phase-related cell cycle molecules in naturally developing hepatocellular carcinoma of Long-Evans Cinnamon rats. Int J Oncol. 2004;24:1205-11 pubmed
    ..These results indicate that cyclin D1 may be involved in the regeneration of hepatocytes rather than hepatocarcinogenesis, while Cdk4 but not Cdk6 may play an important role in the development of HCC...
  38. Márquez Quiñones A, Cipak A, Zarkovic K, Fattel Fazenda S, Villa Treviño S, Waeg G, et al. HNE-protein adducts formation in different pre-carcinogenic stages of hepatitis in LEC rats. Free Radic Res. 2010;44:119-27 pubmed publisher
    ..These results show that lipid peroxidation modification of proteins might be implicated in the first stages of hepatocyte cancer initiation in Long-Evans Cinnamon rats...
  39. Ha Hao D, Merle U, Hofmann C, Wesch H, Doll J, Auburger G, et al. Chances and shortcomins of adenovirus-mediated ATP7B gene transfer in Wilson disease: proof of principle demonstrated in a pilot study with LEC rats. Z Gastroenterol. 2002;40:209-16 pubmed
  40. Nakagawa T, Takeishi S, Kameyama A, Yagi H, Yoshioka T, Moriwaki K, et al. Glycomic analyses of glycoproteins in bile and serum during rat hepatocarcinogenesis. J Proteome Res. 2010;9:4888-96 pubmed publisher
    ..These results suggested that the sorting machinery through fucosylation might be disrupted in the liver with HCC...
  41. Qu L, Liu X, Wu C, Leung L. Hyperthermia decreases GABAergic synaptic transmission in hippocampal neurons of immature rats. Neurobiol Dis. 2007;27:320-7 pubmed
    ..The larger decrease in GABAA-receptor-mediated inhibition during and after hyperthermia, as compared with excitation, may shift the excitation/inhibition balance and contribute to the generation of febrile seizures...
  42. De Bie P, Muller P, Wijmenga C, Klomp L. Molecular pathogenesis of Wilson and Menkes disease: correlation of mutations with molecular defects and disease phenotypes. J Med Genet. 2007;44:673-88 pubmed
    ..As both disorders are characterised by an extensive clinical heterogeneity, we will discus how the underlying genetic defects correlate with the molecular functions of ATP7A and ATP7B and with the clinical expression of these disorders...
  43. Jia G, Takahashi R, Zhang Z, Tsuji Y, Sone H. Aldo-keto reductase 1 family B7 is the gene induced in response to oxidative stress in the livers of Long-Evans Cinnamon rats. Int J Oncol. 2006;29:829-38 pubmed
    ..The results indicate conclusively that in LEC rat liver, akr1b7 might be up-regulated by oxidative stress mediated through NF-kappaB, but not that mediated directly by copper...
  44. Gupta S. Hepatic polyploidy and liver growth control. Semin Cancer Biol. 2000;10:161-71 pubmed
    ..Working models indicate that extensive polyploidy could lead to organ failure, as well as to oncogenesis with activation of precancerous cell clones...
  45. Osanai K, Higuchi J, Oikawa R, Kobayashi M, Tsuchihara K, Iguchi M, et al. Altered lung surfactant system in a Rab38-deficient rat model of Hermansky-Pudlak syndrome. Am J Physiol Lung Cell Mol Physiol. 2010;298:L243-51 pubmed publisher
    ..These results suggest that aberrant lung surfactant secretion may be involved in the pathogenesis of interstitial pneumonia in HPS...
  46. Joseph B, Bhargava K, Malhi H, Schilsky M, Jain D, Palestro C, et al. Sestamibi is a substrate for MDR1 and MDR2 P-glycoprotein genes. Eur J Nucl Med Mol Imaging. 2003;30:1024-31 pubmed
    ..Assessment of P-glycoprotein activity with sestamibi should consider how regulation of ABCB1 and related family members might modulate sestamibi incorporation...
  47. Okumura K, Nakamura K, Hisatomi Y, Nagano K, Tanaka Y, Terada K, et al. Salivary gland progenitor cells induced by duct ligation differentiate into hepatic and pancreatic lineages. Hepatology. 2003;38:104-13 pubmed
    ..In conclusion, the multipotent progenitor cells isolated from the rat salivary gland have characteristics of tissue stem cells and can differentiate into cells of endodermal lineages...
  48. Zhu H, Barr G. The role of AMPA and metabotropic glutamate receptors on morphine withdrawal in infant rats. Int J Dev Neurosci. 2004;22:379-95 pubmed
    ..Thus, for the human infant patient, pharmacotherapies to reduce opiate tolerance and withdrawal should focus on non-NMDA ionotropic and metabotropic receptors...
  49. Fong R, Gonzalez B, Fuentealba I, Cherian M. Role of tumor necrosis factor-alpha in the development of spontaneous hepatic toxicity in Long-Evans Cinnamon rats. Toxicol Appl Pharmacol. 2004;200:121-30 pubmed
    ..Thus, these results suggest a major role for TNF-alpha on the pathogenesis of Cu-induced acute hepatitis in LEC rats...
  50. Chiba M, Itagaki S, Kobayashi M, Hirano T, Iseki K. Down-regulation of intestinal multidrug resistance-associated protein 2 in long-evans cinnamon rats. Drug Metab Pharmacokinet. 2007;22:450-5 pubmed
    ..We found that the expression of multidrug resistance-associated protein 2 (Mrp2) in the jejunum of Long-Evans Cinnamon (LEC) rats, an animal model for Wilson's disease, is decreased...
  51. Leung L, Peloquin P, Canning K. Paired-pulse depression of excitatory postsynaptic current sinks in hippocampal CA1 in vivo. Hippocampus. 2008;18:1008-20 pubmed publisher
    ..PPD of the excitatory sinks provides a measure of population dendritic inhibition in vivo...
  52. Asano A, Tsubomatsu K, Jung C, Sasaki N, Agui T. A deletion mutation of the protein tyrosine phosphatase kappa (Ptprk) gene is responsible for T-helper immunodeficiency (thid) in the LEC rat. Mamm Genome. 2007;18:779-86 pubmed
    ..It is confirmed by this result that the Ptprk gene is responsible for the thid mutation in the LEC rat. Our results further suggest that RPTPkappa plays a critical role in the development of CD4 SP cells in the thymus...
  53. Santon A, Giannetto S, Sturniolo G, Medici V, D Incà R, Irato P, et al. Interactions between Zn and Cu in LEC rats, an animal model of Wilson's disease. Histochem Cell Biol. 2002;117:275-81 pubmed
    ..These results suggest that Zn acts both to compete for absorption on the luminal side of the intestinal epithelium and to induce the synthesis of MT...
  54. Stuchlik A, Rehakova L, Telensky P, Vales K. Morris water maze learning in Long-Evans rats is differentially affected by blockade of D1-like and D2-like dopamine receptors. Neurosci Lett. 2007;422:169-74 pubmed
    ..The results of the present study show a difference in the involvement of D1-like and D2-like receptors in the MWM acquisition...
  55. Sugawara N, Ikeda T, Lai Y, Sugawara C. The effect of subcutaneous tetrathiomolybdate administration on copper and iron metabolism, including their regional redistribution in the brain, in the Long-Evans Cinnamon rat, a bona fide animal model for Wilson's disease. Pharmacol Toxicol. 1999;84:211-7 pubmed
    ..The increased hepatic Fe level should be taken into account when considering side effects of the compound...
  56. Hayashi M, Miyane K, Hirooka T, Endoh D, Higuchi H, Nagahata H, et al. Inhibitory effects of trientine, a copper-chelating agent, on induction of DNA strand breaks in hepatic cells of Long-Evans Cinnamon rats. Biochim Biophys Acta. 2004;1674:312-8 pubmed
    ..The results show that treatment of LEC rats with trientine decreases the number of DNA strand breaks observed, although copper contents remain high in the liver...
  57. Merle U, Encke J, Tuma S, Volkmann M, Naldini L, Stremmel W. Lentiviral gene transfer ameliorates disease progression in Long-Evans cinnamon rats: an animal model for Wilson disease. Scand J Gastroenterol. 2006;41:974-82 pubmed
    ..The aim of this pilot study was to prove the principle of a lentiviral gene transfer in the Long-Evans cinnamon (LEC) rat, an animal model of Wilson disease...
  58. Ahn T, Cho S, Kim D, Jeon B. Absence of nigrostriatal degeneration in LEC rats up to 20 weeks of age. Neurol Res. 2005;27:409-11 pubmed
    ..We studied the pathological changes in the nigrostriatal system of the LEC rat to examine the feasibility of using the LEC rat as a model of neurological Wilson's disease...
  59. Delgado M, Pérez Miguelsanz J, Garrido F, Rodriguez Tarduchy G, Perez Sala D, Pajares M. Early effects of copper accumulation on methionine metabolism. Cell Mol Life Sci. 2008;65:2080-90 pubmed publisher
    ..Taken together, these observations indicate that early copper accumulation alters methionine metabolism with a pattern distinct from that described previously for other liver diseases...
  60. Sone H, Li Y, Ishizuka M, Aoki Y, Nagao M. Increased mutant frequency and altered mutation spectrum of the lacI transgene in Wilson disease rats with hepatitis. Cancer Res. 2000;60:5080-6 pubmed
    ..These findings suggest that the increase in mutant frequencies and/or the specific type of mutation according to copper accumulation play a crucial role in hepatocarcinogenesis in LEC rats...
  61. Santon A, Albergoni V, Sturniolo G, Irato P. Evaluation of MT expression and detection of apoptotic cells in LEC rat kidneys. Biochim Biophys Acta. 2004;1688:223-31 pubmed
    ..These findings suggest that, in LEC rats, Zn may contribute to cytoprotection through the regulation of MT expression which may provide a cellular defence strategy in response to DNA damage...
  62. Imai K, Sato M, Kojima N, Miura M, Sato T, Sugiyama T, et al. Storage of lipid droplets in and production of extracellular matrix by hepatic stellate cells (vitamin A-storing cells) in Long-Evans cinnamon-like colored (LEC) rats. Anat Rec. 2000;258:338-48 pubmed
    ..Our present data are consistent with our previous hypothesis that there is an antagonistic relationship between the storage of vitamin A and the production of collagen in stellate cells...
  63. Ikeda H, Nishi S, Sakai M. Transcription factor Nrf2/MafK regulates rat placental glutathione S-transferase gene during hepatocarcinogenesis. Biochem J. 2004;380:515-21 pubmed
    ..These results indicate that the Nrf2/MafK heterodimer regulates GST-P gene expression during early hepatocarcinogenesis and in hepatoma cells...
  64. Samuele A, Mangiagalli A, Armentero M, Fancellu R, Bazzini E, Vairetti M, et al. Oxidative stress and pro-apoptotic conditions in a rodent model of Wilson's disease. Biochim Biophys Acta. 2005;1741:325-30 pubmed
    ..Hence, we assume that enhanced oxidative stress may play a central role in the cell degeneration in WD, at the main sites of copper deposition, with discrete pro-apoptotic conditions developing in distal areas...
  65. Du C, Fujii Y, Ito M, Harada M, Moriyama E, Shimada R, et al. Dietary polyunsaturated fatty acids suppress acute hepatitis, alter gene expression and prolong survival of female Long-Evans Cinnamon rats, a model of Wilson disease. J Nutr Biochem. 2004;15:273-80 pubmed
  66. Hayashi M, Nagata A, Endoh D, Arikawa J, Okui T. High sensitivity of thymocytes of LEC strain rats to induction of apoptosis by X-irradiation. J Vet Med Sci. 2002;64:597-601 pubmed
    ..The present results showed high sensitivities of thymocytes of LEC rats to induction of apoptosis during in vitro cultivation and by X-irradiation...
  67. Kinebuchi M, Matsuura A, Ohya K, Abo W, Kitazawa J. Contribution of Va24Vb11 natural killer T cells in Wilsonian hepatitis. Clin Exp Immunol. 2005;139:144-51 pubmed
    ..Furthermore, liver lymphocytes in the acute jaundiced LEC rats with CD161highalphabetaTCRbright NKT cells had significant and CD1d-specific cytotoxic activity...
  68. Iwata R, Sasaki N, Agui T. Contiguous gene deletion of Ptprk and Themis causes T-helper immunodeficiency (thid) in the LEC rat. Biomed Res. 2010;31:83-7 pubmed
    ..The result suggests that the deficiency of both genes is responsible for the thid mutation, and that both genes are indispensable for the development of SP cells from DP cells in the thymus...
  69. Dotigny F, Ben Amor A, Burke M, Vaucher E. Neuromodulatory role of acetylcholine in visually-induced cortical activation: behavioral and neuroanatomical correlates. Neuroscience. 2008;154:1607-18 pubmed publisher
  70. Santollo J, Wiley M, Eckel L. Acute activation of ER alpha decreases food intake, meal size, and body weight in ovariectomized rats. Am J Physiol Regul Integr Comp Physiol. 2007;293:R2194-201 pubmed
    ..Taken together, our findings demonstrate that selective activation of ER alpha decreases food intake, body weight, and meal size in the ovariectomized rat...
  71. Kasai N. Possible role of zinc and iron in the development of hepatic abnormality in Long-Evans Cinnamon rats. J Gastroenterol Hepatol. 2007;22:147-9 pubmed
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    ..Engraftment of transplanted cells, which express atp7b mRNA, repopulated the recipient liver with normal functional capacity...
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    ..We evaluated the feasibility of the Wig rat for an animal model of human attention deficit hyperactive disorder (ADHD)...
  74. Suzuki K, Takenaka J, Ogra Y. Identification of the zinc-binding protein specifically present in male rat liver as carbonic anhydrase III. Chem Biol Interact. 1999;122:185-97 pubmed
    ..The concentration of CAIII was decreased specifically by repeated injections of cupric ions without the Cu,Zn-SOD concentration being affected...
  75. Santon A, Irato P, Medici V, D Incà R, Albergoni V, Sturniolo G. Effect and possible role of Zn treatment in LEC rats, an animal model of Wilson's disease. Biochim Biophys Acta. 2003;1637:91-7 pubmed
  76. Klein D, Arora U, Lichtmannegger J, Finckh M, Heinzmann U, Summer K. Tetrathiomolybdate in the treatment of acute hepatitis in an animal model for Wilson disease. J Hepatol. 2004;40:409-16 pubmed
    ..Here, we investigate the potential use of TTM in treating the acute hepatic copper toxicosis in Long-Evans Cinnamon (LEC) rats, an authentic model for Wilson disease...
  77. Irani A, Malhi H, Slehria S, Gorla G, Volenberg I, Schilsky M, et al. Correction of liver disease following transplantation of normal rat hepatocytes into Long-Evans Cinnamon rats modeling Wilson's disease. Mol Ther. 2001;3:302-9 pubmed
    ..These data indicate that liver repopulation with functionally intact cells can reverse pathophysiological perturbations and cure Wilson's disease...
  78. Shishido N, Nakayama K, Takazawa A, Ohyama T, Nakamura M. Cu-metallothioneins (Cu(I)8-MTs) in LEC rat livers 13 weeks after birth still act as antioxidants. Arch Biochem Biophys. 2001;387:216-22 pubmed
    ..Cu2+-catalyzed oxidation of DNA was strongly inhibited both in the presence of Cu-unsaturated MTs and GSH. The results suggest that Cu(I)8-MTs from LEC rat livers just before hepatitis still act as antioxidants...
  79. Kawano H, Takeuchi Y, Yoshimoto K, Matsumoto K, Sugimoto T. Histological changes in monoaminergic neurons of Long-Evans Cinnamon rats. Brain Res. 2001;915:25-31 pubmed
    ..These results suggest that age-dependent changes in copper concentrations of Long-Evans Cinnamon rats were related to changes in monoaminergic neuron systems...