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DIT sugar and energy
| Question | Answer |
|---|---|
| glucose to glucose 6P | hexokinase/glucokinase |
| fructose 6P to fructose 1,6 bisphosphonate | PFK1 |
| PEP to pyruvate | pyruvate kinase |
| 3 important enzymes of glycolysis | hexokinase/glucokinase, PFK1, pyruvate kinase |
| glycogen phosphorylase deficiency | McArdles |
| glucose 6P def | Von Gierke's |
| Lactic acidosis, hyperlipidemia, hyperuricemia | Von Gierke's |
| alpha 1,6 glucosidase deficiency | Cori |
| alpha 1,4, glucosidase deficiency | Pompe |
| cardiomegaly | Pompe (infantile) |
| diphragm weakness causing respiratory failure | Pompe (adult) |
| increasd glycogen in liver, severe fasting hypoglycemia | von gierke |
| hepatomegaly, hypoglycemia, hyperlipidemia (nL kidneys, lactate, and uric acid) | Cori |
| Painful muscle cramps, myoglobinuria with strenuous exercise | McArdle |
| sever hepatosplenomegaly, enlarged kidneys | Von Gierke |
| m biopsy shows elevated glycogen, fructose 6P, and decreased pyruvate. | deficiency in PFK1 |
| what enzyme is responsible for increasing intracellular levels of fru 2,6 bisP | PFK2 |
| what enzyme is responsible for decreasing intracellular levels of fru2,6bisP | FBP2 |
| how will levels of fru2,6bisP change in the liver and muscle during a sympathetic response? | decrease in the liver, increase in the muscle |
| common cause of glycolytic enzyme def | pyruvate kinase deficiency, phosphoglucose isomerase deficiency |
| clinical presentation of glycolytic enzyme def | hemolytic anemia due to inability to maintain Na/K ATPase causing RBC swelling and lysis |
| Why are alanine and glutamine found in such high concentrations in the blood | They are the 2 major carriers of nitrogen from tissues |
| What is required by all aminotransferases | pyridoxal phosphate (a B6 derivative) |
| alanine + alphaKG ---> glutamate + pyruvate | ALT |
| glutamate + oxaloacetate ---> alpaKG + aspartate | AST |