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Alteration of mitochondrial function in the livers of mice with glycogen branching enzyme deficiency
Autor:
Jordi Duran, Giorgia Testoni, Jerzy Duszyński, Malgorzata Bejtka, Dominika Malinska, Joan J. Guinovart
Publikováno v:
Biochimie. 186
Glycogen storage disease type IV (GSD IV) is caused by mutations in the glycogen branching enzyme gene (GBE1) that lead to the accumulation of aberrant glycogen in affected tissues, mostly in the liver. To determine whether dysfunctional glycogen met