1. Rate Determining Enzyme of Glycolysis
  2. Rate Determining Enzyme of Gluconeogenesis
    Fructose 1,6 bisphosphatase
  3. Rate Determining Enzyme of TCA cycle
    Isocitrate dehydrogenase
  4. Rate Determining Enzyme of Glycogen synthesis
    Glycogen synthase
  5. Rate Determining Enzyme of Glycogenolysis
    Glycogen phosphorylase
  6. Rate Determining Enzyme of HMP shunt
    Glucose-6-phosphate dehydrogenase
  7. Rate Determining Enzyme of De novo pyrimidine synthesis
    Carbamoyl phosphate synthetase II
  8. Rate Determining Enzyme of De nova purine synthesis
    Glutamine-PRPP amidotransferase
  9. Rate Determining Enzyme of Urea cycle
    carbamoyl phosphate synthetase I
  10. Rate Determining Enzyme of Fatty Acid Synthesis
    Acetyl-CoA carboxylase
  11. Rate Determining Enzyme of Fatty acid oxidation
    Carnitine acyltransferase I
  12. Rate Determining Enzyme of Ketogenesis
    HMG-CoA synthase
  13. Rate Determining Enzyme of Cholesterol synthesis
    HMG-CoA reductase
  14. A child who fails to track objects or to develop a social smile with infantile cataracts will produce what in their blood and urine?
    Galactose, due to galactokinase deficiency. AR. relatively mild.
  15. A child with failure to thrive, jaundice, hepatomegaly, infantile cataracts, mental retardation is missing what enzyme?
    galactose-1-phosphate uridyltransferase. AR.
  16. What enzyme is deficient in von Gierke's disease?
  17. What enzyme is deficient in Pompe's disease?
    lysosomal alpha-1,4-glucosidase
  18. What enzyme is deficient in Cori's disease?
    Debranching enzyme
  19. What enzyme is deficient in McArdle's disease?
    Skeletal muscle glycogen phosphorylase
  20. When fructose appear in urine, what enzyme is deficient?
  21. Hypoglycemia, jaundice, cirrhosis, and vomiting can be seen in patients with this deficiency which decreases available phosphate:
    Aldolase B - fructose intolerance
  22. What two enzymes are active during a fast?
    Fructose bisphosphate 2 & Fructose-1,6-bisphosphatase
  23. What two enzymes are active in the fed state?
    PFK-1 & PFK-2
  24. Patients with chronic granulomatous disease are at a risk for which organisms?
    Organisms that are catalase positive: S. aureus, Asperigillus
  25. Fava beans, sulfonamides, primaquine, and antituberculosis drugs cause hemolytic anemia in which patients?
    G6PD deficient patients due to the lack of NADPH to detoxify free radicals and peroxides.
  26. Glucokinase vs. Hexokinase - Which can be induced by insulin, has a high Vmax, and is only found in the liver?
  27. What two enzymes are associated with hemolytic anemia due to an inability to maintain activity of the Na/K ATPase pump?
    pyruvate kinase (95%), phosphoglucose isomerase (4%)
  28. A patient with vomiting, rice water stools, and garlic breath is intoxicated by what and why?
    Arsenic due to it's inhibition of lipoic acid which is needed by pyruvate dehydrogenase
  29. What five cofacts are needed by pyruvate dehydrogenase complex?
    Thiamine, Riboflavin, Niacin, Pantothenate, & Lipoic acid
  30. What are the two purely ketogenic amino acids?
    Lysine & Leucine
  31. What can high fat content, Lysine & Leucine be used to treat?
    Pyruvate dehydrogenase deficiency (congenital or acquired)
  32. What are the four irreversible enzymes for gluconeogenesis?
    Pyruvate carboxylase, PEP carboxykinase, Fructose 1-6 bisphophatase, Glucose -6-phosphatase
  33. Rotenone, CN, antimycin A, CO are all?
    Electron transport inhibitors
  34. What is an ATPase inhibitor?
  35. 2,4 DNP and aspirin are both?
    uncoupling agents of the electron transport chain
  36. What creates a osmotic pressure that causes water to enter the cell and produce osmotic damage?
    Sorbitol, made from glucose via Aldose reductase
  37. What can be given to decrease ammonia levels?
    Benzoate or phenylbutyrate
  38. A patient who presents with tremor, slurring of speech, somnolence, vomiting, cerebral edema, and blurring of vision is suffering an intoxication of?
  39. A patient with an increase of orotic acid in blood and urine, decrease BUN and symptoms of hyperammonemia is deficient in what enzyme?
    ornithine transcarbamoylase. X linked recessive
Card Set