Hepatic Carbohydrate Response Element Binding Protein Activation Limits Nonalcoholic Fatty Liver Disease Development in a Mouse Model for Glycogen Storage Disease Type 1a.

ABSTRACT:

Background and aims

Glycogen storage disease (GSD) type 1a is an inborn error of metabolism caused by defective glucose-6-phosphatase catalytic subunit (G6PC) activity. Patients with GSD 1a exhibit severe hepatomegaly due to glycogen and triglyceride (TG) accumulation in the liver. We have shown that the activity of carbohydrate response element binding protein (ChREBP), a key regulator of glycolysis and de novo lipogenesis, is increased in GSD 1a. In the current study, we assessed the contribution of ChREBP to nonalcoholic fatty liver disease (NAFLD) development in a mouse model for hepatic GSD 1a.

Approach and results

Liver-specific G6pc-knockout (L-G6pc^-/- ) mice were treated with adeno-associated viruses (AAVs) 2 or 8 directed against short hairpin ChREBP to n

SUBMITTER: Lei Y 

PROVIDER: S-EPMC7702155 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature