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Metabolic reasoning

Whole-map thinking. Each case is a chain of cause → effect that ends in the labs: why DKA has ketones and hyperglycemia, why a drinker goes hypoglycemic, why an infant with MCAD deficiency spills no ketones. Walk through the chain on the map, then order it yourself, answer the why questions, or diagnose from the labs.

Case
The big map. Highlighted boxes are the parts of metabolism the current step is about. Scroll sideways when zoomed.
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GlycogenGlycogenGlucoseGlucoseGalactoseGalactoseFructoseFructoseGlucose-6-phosphateGlucose-6-phosphateRibose-5-P + NADPHRibose-5-P + NADPHFructose-6-phosphateFructose-6-phosphateFructose-1,6-bisPFructose-1,6-bisPG3P / DHAPG3P / DHAPGlycerol (fat)Glycerol (fat)PhosphoenolpyruvatePhosphoenolpyruvatePyruvatePyruvateLactateLactateAlanine (amino acids)Alanine (amino acids)Acetyl-CoAAcetyl-CoAOxaloacetateOxaloacetateCitrateCitrateTCA cycle → NADH/FADH₂TCA cycle → NADH/FADH₂ETC → ATPETC → ATPFatty acidsFatty acidsTriacylglycerolTriacylglycerolKetone bodiesKetone bodiesCholesterolCholesterolEthanolEthanolGlycogenolysis (phosphorylase)Hexokinase / glucokinaseLeloir pathway (GALT)HMP shunt (G6PD)GlycolysisPFK-1 ★ / FBPase-1AldolaseFructokinase → aldolase B(bypasses PFK-1)Glycerol kinase (liver)Hormone-sensitive lipaseLower glycolysis (2 ATP, 2 NADH)Pyruvate kinaseALT (glucose-alanine cycle)LDH (anaerobic)PDH (irreversible; thiamine)ADH → ALDH (2 NADH)Pyruvate carboxylase (biotin; +acetyl-CoA)Citrate synthaseCitrate synthaseTCA cycleOXPHOSATP-citrate lyase → ACC → FAS(needs NADPH)Hormone-sensitive lipaseHMG-CoA synthase / lyase (liver)HMG-CoA reductase (cytosol)

Vomiting and hypoglycemia after the first fruit

A 6-month-old was thriving on breast milk and formula. Two weeks after starting fruit purées and juice she has vomiting, lethargy, sweating and irritability after meals, with jaundice and an enlarged liver.

Glucose (post-prandial)38 mg/dL
Urine reducing substancespositive+
Urine glucose oxidasenegative
AST/ALT, bilirubin
Phosphate
Uric acid

Hereditary fructose intolerance (aldolase B deficiency)

Fructose-1-phosphate traps the liver's phosphate; without Pi there is no ATP, and without ATP neither glycogenolysis nor gluconeogenesis can run.

Condition card: Hereditary fructose intolerance (secondary hyperuricemia) →Pathway map →

  1. Sucrose/fruit fructose → GLUT-5 → portal blood → liver takes 90% on first pass.

Pathways involved: Fructose metabolism, Glycogen synthesis & breakdown, Gluconeogenesis. Drill them one at a time in Metabolic pathways.

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