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20 Cards in this Set
- Front
- Back
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Galactose --> Galactose 1-phosphate
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Galactokinase (mild galactosemia)
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Galactose-1-phosphate --> Glucose-1-phosphate
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Galactose-1-phosphate uridyltransferase (severe galactosemia)
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Glucose --> Glucose-6-phosphate
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Hexokinase/Glucokinase
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Glucose-6-phosphate--> 6-phosphogluconolactone
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G6PD deficiency
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Glucose-6-phosphate --> Glucose
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Glucose-6-phosphatase (von Gierke's)
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Ribulose-5-phosphate --> Fructose-6-phosphate
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Transketolase + TPP (thiamine)
** HMP shunt produces NADPH for FA + steroid biosynth |
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Fructose-6-phosphate --> Fructose-1,6,-bisphosphate
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PFK-1
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Fructose-1,6-bisphophate --> Fructose-6-phsophate
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Fructose-1,6-bisphosphatase
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Fructose --> F1P
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Fructokinase (essential fructosuria)
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F1P --> DHAP or Glyceraldehyde
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Aldolase B (fructose intolerance)
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Phosphoenolpyruvate (PEP) --> Pyruvate
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Pyruvate kinase
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Pyruvate --> Acetyl CoA
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Pyruvate dehydrogenase + TPP
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HMG CoA --> Mevalonate
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HMG CoA reductase
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Pyruvate --> OAA
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Pyruvate carboxylase + Biotin
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OAA --> Phosphoenolpyruvate (PEP)
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PEP carboxykinase
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OAA --> Citrate
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Citrate synthase
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Isocitrate --> alpha-ketoglutarate
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Isocitrate dehydrogenase
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Alpha-ketoglutarate --> Succinyl CoA
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Alpha-ketoglutarate dehydrogenase + TPP
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Ornithine --> Citrulline
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Ornithine transcarbamoylase + Carbamoyl phosphate
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Odd Chain fatty acids
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OCFA's --> Propionyl CoA
Propionyl CoA + Biotin --> Methylmalonyl CoA ISOMERIZATION: Methylmalonyl CoA + B12 --> Succinyl CoA (TCA cycle) - defect here causes methylamolnic aciduria |
