Mutation of Npc1 gene causes abnormal lipid metabolism to induce apoptosis of renal cells and promote fibrosis |
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| Authors: | NIU Li-dan YANG Ji-chao GUAN Li-hong DU Jiang QIAO Liang LIU Yan-li LIN Jun-tang |
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| Institution: | 1. School of Biomedical Engineering, Xinxiang Medical University, Xinxiang 453003, China;
2. Stem Cell and Biotherapy Technology Research Center, Xinxiang Medical University, Xinxiang 453003, China;
3. Institute of Mental and Neurology, Xinxiang Medical University, Xinxiang 453003, China;
4. College of Life Science and Technology, Xinxiang Medical University, Xinxiang 453003, China |
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| Abstract: | AIM: To investigate the dysfunction of renal cell and tissue in Npc1 mutant mice, in order to provide support for the treatment of Niemann-Pick disease type C1 (NPC1) patients.METHODS: The kidneys of wild-type (Npc1+/+) and Npc1 mutant (Npc1-/-) mice on postnatal day 60 were isolated. HE staining was performed to examine the morphological changes of the renal tissues. Oil red O staining was used to examine the lipid deposition in the renal tissues. The apoptosis of the renal cells was detected by TUNEL staining. The expression of apoptosis-related proteins in the renal tissue was determined by Western blot, and immunofluorescence was performed to examine the expression of α-smooth muscle actin (α-SMA) and vimentin in the renal tissues. RESULTS: Compared with Npc1+/+ mice, the morphological observation showed obvious vacuoles and no lipid deposition in the renal tissue of Npc1-/- mice. Subsequently, TUNEL staining showed significant increase in the apoptotic cells in the renal tissue of Npc1-/- mice (P<0.01), and the expression levels of Bax and Bad were up-regulated in the renal tissues of Npc1-/- mice (P<0.01), but Bcl-2 was down-regulated (P<0.05). Furthermore, the expression of α-SMA and vimentin was significantly up-regulated in the renal tissues of Npc1-/- mice (P<0.01). CONCLUSION: Npc1 gene mutation causes abnormal lipid metabolism in the renal cells, which induces the apoptosis of renal cells and promotes the fibrosis of renal tissue. |
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| Keywords: | Niemann-Pick disease type C1 Lipid metabolism Apoptosis Fibrosis Npc1 gene |
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