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A proteomic approach to identify biomarkers of Glycogen Storage Disease type 1a progression in mouse livers: evidence for metabolic reprogramming, tissue inflammation and macrophage polarization

ABSTRACT: Glycogen storage disease type 1a (GSD-1a) is an autosomal recessive disorder caused by mutations in the catalytic subunit of the glucose-6-phosphatase-alpha (G6Pase-α). The current treatment of GSD-1a is based on the control of symptomatic hypoglycemia. Long-term complications such as renal failure and development of hepatocellular adenoma/carcinoma develop in the majority of patients. The aim of this study was to determine the proteomic expression changes in the liver of LS-G6pc-/- mice, a mouse model of GSD-1a, in comparison with wild type mice to identify potential biomarkers of the pathophysiology of the affected liver.

INSTRUMENT(S): LTQ Orbitrap Velos

ORGANISM(S): Mus Musculus (mouse)

TISSUE(S): Liver

SUBMITTER: Andrea Petretto

LAB HEAD: Andrea Petretto

PROVIDER: PXD011561 | Pride | 2019-11-25

REPOSITORIES: Pride

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A proteomic approach to identify biomarkers of Glycogen Storage Disease type 1a progression in mouse livers: evidence for metabolic reprogramming, tissue inflammation and macrophage polarization

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