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Glycogen storage disease type Ia (von Gierke)

G6PCOMIM 232200do(g6pase_activity ↓)

loss of glucose-6-phosphatase -> impaired hepatic glucose output; G6P shunted to lactate.
plasma glucose concentration ↓HPO: HypoglycemiaHP:0001943
comparative-statics (steady-state) net: ?
hepatic glucose-6-phosphatase activity (G6PC) ↓ →(+) plasma glucose concentration ↓
hepatic glucose-6-phosphatase activity (G6PC) ↓ →(+) hepatic gluconeogenesis rate (lactate/glycerol/amino acids -> glucose-6-phosphate) ↓ ↠(production +) plasma glucose concentration ↓
hepatic glucose-6-phosphatase activity (G6PC) ↓ →(+) hepatic glycogenolysis rate (glycogen -> glucose-6-phosphate) ↓ ↠(production +) plasma glucose concentration ↓
hepatic glucose-6-phosphatase activity (G6PC) ↓ →(+) hepatic gluconeogenesis rate (lactate/glycerol/amino acids -> glucose-6-phosphate) ↓ →(+) hepatic glucose production (glycogenolysis + gluconeogenesis) ↓ →(+) plasma glucose concentration ↓
hepatic glucose-6-phosphatase activity (G6PC) ↓ →(+) hepatic glycogenolysis rate (glycogen -> glucose-6-phosphate) ↓ →(+) hepatic glucose production (glycogenolysis + gluconeogenesis) ↓ →(+) plasma glucose concentration ↓
plasma lactate concentration ↑HPO: Lactic acidosisHP:0003128
comparative-statics (steady-state) net:
hepatic glucose-6-phosphatase activity (G6PC) ↓ →(-) plasma lactate concentration ↑
↑ increased   ↓ decreased   ? magnitude-dependent (SCC). →(+) positive edge, →(−) negative, cross-scale constitutive lift. Endophenotypes are real HPO directional terms. Draft for domain review.
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