Renal function and pathological changes in Niemann-Pick disease type C1 mice / 中国病理生理杂志

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 con-tent of important indicators in the serum including ALT , AST, LDH, urea, UA and Cr.Furthermore,β-galactosidase stai-ning 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-associatedβ-galacto-sidase 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:Muta-tion 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 .