Negative feedback loop of cholesterol regulation is impaired in the livers of patients with Alagille syndrome.

Yuki Miyahara,Kazuhiko Bessho,Hiroki Kondou,Yasuhiro Hasegawa,Kie Yasuda,Shinobu Ida,Yoshiyuki Ihara,Koichi Mizuta,Yoko Miyoshi,Keiichi Ozono

Clinica Chimica Acta（2015）

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摘要

•Liver cholesterol content of patients with Alagille syndrome (AGS) was increased.•Upregulation of LDLR, SR-BI and HMGCR might contribute to high cholesterol content.•Maturation of SREBP2 was not suppressed despite high cholesterol content in AGS.•Negative feed-back mechanism of cholesterol regulation was impaired in AGS children.

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关键词

AGS,BA,TC,TBA,LDLR,HDL,SR-BI,HMGCR,FXR,LXRα,CYP7A1,SHP,SREBP2

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