Renal function and pathological changes in Niemann-Pick disease type C1 mice |
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Authors: | LIU Yan-li QIAO Liang ZHANG Jin-zhu YANG Fen YAN Yan YAN Xin LIN Jun-tang |
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Institution: | 1. College of Life Science and Technology, Xinxiang Medical University, Xinxiang 453003, China;
2. Henan Key Laboratory of Medical Tissue Regeneration, Xinxiang 453003, China |
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Abstract: | AIM: To investigate the renal function and pathological changes in Npc1 mutant (Npc1-/-) mice. METHODS: Different genotypes of Niemann-Pick disease type C1 (Npc1) mice were identified by PCR. Subsequently, the renal function of Npc1-/- and Npc1+/+ mice at postnatal day 60 (P60) was evaluated by measuring the activity and content of important indicators in the serum including ALT, AST, LDH, urea, UA and Cr. Furthermore, β-galactosidase staining and Masson staining were performed to examine the aging and fibrosis of the renal tissues, respectively. RESULTS: Compared with the Npc1+/+ mice, the body weight and kidney weight had a significant reduction (P<0.01) in the Npc1-/- mice. The results of hepatic and renal functions showed that the activities of ALT, AST and LDH, and contents of urea, UA and Cr had marked increases (P<0.05) in the Npc1-/-mice. Moreover, the results of senescence-associated β-galactosidase staining in the renal tissues demonstrated accelerated aging in the Npc1-/- mice (P<0.01), and these results were confirmed by Masson staining, which clearly showed the formation of collagen fibers (P<0.01).CONCLUSION: Mutation of the Npc1 gene results in abnormal lipid metabolism, which accelerates kidney senescence by promoting fibrosis in the renal tissue and subsequently causes reduction in renal function. |
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Keywords: | Niemann-Pick disease type C1 Kidney Fibrosis Npc1 gene |
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