Liver Glycogen Phosphorylase Deficiency Leads to Profibrogenic Phenotype in a Murine Model of Glycogen Storage Disease Type VI

Lane H. Wilson, Jun‐Ho Cho, Ana Estrella, Joan A. Smyth, Rong Wu, Tayoot Chengsupanimit, Laurie M. Brown, David A. Weinstein, Young Mok Lee – 24 September 2019 – Mutations in the liver glycogen phosphorylase (Pygl) gene are associated with the diagnosis of glycogen storage disease type VI (GSD‐VI). To understand the pathogenesis of GSD‐VI, we generated a mouse model with Pygl deficiency (Pygl−/−). Pygl−/− mice exhibit hepatomegaly, excessive hepatic glycogen accumulation, and low hepatic free glucose along with lower fasting blood glucose levels and elevated blood ketone bodies.

RPB5‐Mediating Protein Promotes Cholangiocarcinoma Tumorigenesis and Drug Resistance by Competing With NRF2 for KEAP1 Binding

Zheng‐Hua Wan, Tian‐Yi Jiang, Yuan‐Yuan Shi, Yu‐Fei Pan, Yun‐Kai Lin, Yun‐Han Ma, Chun Yang, Xiao‐Fan Feng, Li‐Feng Huang, Xiao‐Ni Kong, Zhi‐Wen Ding, Ye‐Xiong Tan, Li‐Wei Dong, Hong‐Yang Wang – 21 September 2019

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