How does von Gierk’s disease lead to lactic acidosis & ketosis at the same time?
in von Gierk’s disease, there is no Glu-6-P-ase enzyme, so, in fasting, glycogenolysis yields g-6-p only, there is increased lipolysis (due to low insulin) with increase production of acetyle coA from excessive F.A oxidation to produce energy due to lack of free glucose to use for energy production. High acetyl co form ketone bodies because cannot enter TCA cycle (no oxalo-acetate because PC is inactive) so, it only form k.b.
