Dietary exacerbation of metabolic stress leads to accelerated hepatic carcinogenesis in glycogen storage disease type Ia
M Gjorgjieva, J Calderaro, L Monteillet, M Silva… - Journal of …, 2018 - Elsevier
Background & Aims Glycogen storage disease type Ia (GSDIa) is a rare genetic disease
associated with glycogen accumulation in hepatocytes and steatosis. With age, most adult
patients with GSDIa develop hepatocellular adenomas (HCA), which can progress to
hepatocellular carcinomas (HCC). In this study, we characterized metabolic reprogramming
and cellular defense alterations during tumorigenesis in the liver of hepatocyte-specific
G6pc deficient (L. G6pc−/−) mice, which develop all the hepatic hallmarks of GSDIa …
associated with glycogen accumulation in hepatocytes and steatosis. With age, most adult
patients with GSDIa develop hepatocellular adenomas (HCA), which can progress to
hepatocellular carcinomas (HCC). In this study, we characterized metabolic reprogramming
and cellular defense alterations during tumorigenesis in the liver of hepatocyte-specific
G6pc deficient (L. G6pc−/−) mice, which develop all the hepatic hallmarks of GSDIa …