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Home > Archive>Volume 24, Issue 11, 2014 >1-5. DOI:10.3969.j.issn.1671.7856.2014.011.001
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To observe the NTBC dependence of Fah-knockout mice
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    Abstract:

    Objective To observe the NTBC dependence of Fah-knockout mice and study the biological characteristics in order to use the model more effectively. Methods Examine the progressive changes in body weight, survival time, liver pathology and serological markers after the NTBC withdrawal. Results After removing of NTBC, Fah-knockout mice lost their body weight gradually, and finally died in 5 to 7 weeks, along with increased serum ALT, AST levels and deformation of the hepatocytes. Conclusions Fah-knockout mice have a strong drug dependence of NTBC and could be the ideal model to hereditary tyrosinemia type I and other liver injury.

    Reference
    [1] Grompe M, Al-Dhalimy M, Finegold M, et al. Loss of fumarylacetoacetate hydrolase is responsible for the neonatal hepatic dysfunction phenotype of lethal albino mice[J]. Genes & development, 1993, 7(12a): 2298-2307.
    [2] Orejuela D, Jorquera R, Bergeron A, et al. Hepatic stress in hereditary tyrosinemia type 1 (HT1) activates the AKT survival pathway in thefah-knockout mice model[J]. Journal of hepatology, 2008, 48(2): 308-317.
    [3] Qi Z, Wang X, Wei H, et al. Infiltrating neutrophils aggravate metabolic liver failure in fah-deficient mice[J]. Liver International, 2014,DOI: 10.1111/liv.12594.
    [4] Masurel-Paulet A, Poggi-Bach J, Rolland M O, et al. NTBC treatment in tyrosinaemia type I: long-term outcome in French patients[J]. Journal of inherited metabolic disease, 2008, 31(1): 81-87.
    [5] Su B L, Liu C C, Xiang D, et al. Xeno-repopulation of Fah-/- Nod/Scid mice livers by human hepatocytes[J]. Science China Life Sciences, 2011, 54(3): 227-234.
    [6] Buitrago-Molina L E, Marhenke S, Longerich T, et al. The degree of liver injury determines the role of p21 in liver regeneration and hepatocarcinogenesis in mice[J]. Hepatology, 2013, 58(3): 1143-1152.
    [7] Alison M R, Lovell M J. Liver cancer: the role of stem cells[J]. Cell proliferation, 2005, 38(6): 407-421.
    [8] Kubo S, Sun M, Miyahara M, et al. Hepatocyte injury in tyrosinemia type 1 is induced by fumarylacetoacetate and is inhibited by caspase inhibitors[J]. Proceedings of the National Academy of Sciences, 1998, 95(16): 9552-9557.
    [9] 苏宝亮, 刘长城, 李帅, 等. Fah 基因剔除小鼠在肝细胞移植中的应用及其病理学变化[J]. 中国药科大学学报, 2009 9, 40(6): 553 - 558.
    [10] Wilber A, Wangensteen K J, Chen Y, et al. Messenger RNA as a Source of Transposase for Sleeping Beauty Transposon-mediated Correction of Hereditary Tyrosinemia Type I[J]. Molecular Therapy, 2007, 15(7): 1280-1287.
    [11] 张洪, 鲍波. 浅谈国内BALB/c小鼠及KM小鼠的基本生物学特性[J]. 中国实用医药, 2010 (3): 252-254.
    [12] 魏立,张丽娟, 张跃新. 肝病患者血清学指标与肝纤维化程度的关系[J]. 标记免疫分析与临床, 2009, 16(4): 205-208.
    [13] Fernandes J, Saudubray J M, Van Den Berghe G, et al. Inborn metabolic diseases: diagnosis and treatment[M]. Springer, 2006:233-243.
    [14] 杨楠, 韩连书, 叶军, 等. 尼替西农治疗2例酪氨酸血症Ⅰ型的效果分析并文献复习[J]. 临床儿科杂志, 2012, 29(12): 1178-1181.
    [15] Nieto N, Friedman SL, Cederbaum Al. Stimulation and proliferation of primary rat hepaticstellate cells by cytochrome P450 2E1-derived reactive oxygen species[J]. Hepatology,2002, 35 (1): 62-73.
    [16] Nieto N. Ethanol and fish oil induce NFkappaB transactivation of the collagen alpha2(l)promoter through lipid peroxidation-driven activation of the PKC-PI3K-Akt pathway [J].Hepatology, 2007, 45 (6): 1433-1445.
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  • Revised:October 22,2014
  • Online: December 05,2014
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