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Rate Determining Enzyme of Glycolysis
PFK-1
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Rate Determining Enzyme of Gluconeogenesis
Fructose 1,6 bisphosphatase
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Rate Determining Enzyme of TCA cycle
Isocitrate dehydrogenase
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Rate Determining Enzyme of Glycogen synthesis
Glycogen synthase
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Rate Determining Enzyme of Glycogenolysis
Glycogen phosphorylase
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Rate Determining Enzyme of HMP shunt
Glucose-6-phosphate dehydrogenase
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Rate Determining Enzyme of De novo pyrimidine synthesis
Carbamoyl phosphate synthetase II
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Rate Determining Enzyme of De nova purine synthesis
Glutamine-PRPP amidotransferase
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Rate Determining Enzyme of Urea cycle
carbamoyl phosphate synthetase I
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Rate Determining Enzyme of Fatty Acid Synthesis
Acetyl-CoA carboxylase
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Rate Determining Enzyme of Fatty acid oxidation
Carnitine acyltransferase I
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Rate Determining Enzyme of Ketogenesis
HMG-CoA synthase
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Rate Determining Enzyme of Cholesterol synthesis
HMG-CoA reductase
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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.
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A child with failure to thrive, jaundice, hepatomegaly, infantile cataracts, mental retardation is missing what enzyme?
galactose-1-phosphate uridyltransferase. AR.
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What enzyme is deficient in von Gierke's disease?
glucose-6-phosphate
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What enzyme is deficient in Pompe's disease?
lysosomal alpha-1,4-glucosidase
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What enzyme is deficient in Cori's disease?
Debranching enzyme
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What enzyme is deficient in McArdle's disease?
Skeletal muscle glycogen phosphorylase
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When fructose appear in urine, what enzyme is deficient?
Fructokinase
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Hypoglycemia, jaundice, cirrhosis, and vomiting can be seen in patients with this deficiency which decreases available phosphate:
Aldolase B - fructose intolerance
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What two enzymes are active during a fast?
Fructose bisphosphate 2 & Fructose-1,6-bisphosphatase
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What two enzymes are active in the fed state?
PFK-1 & PFK-2
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Patients with chronic granulomatous disease are at a risk for which organisms?
Organisms that are catalase positive: S. aureus, Asperigillus
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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.
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Glucokinase vs. Hexokinase - Which can be induced by insulin, has a high Vmax, and is only found in the liver?
Glucokinase
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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%)
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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
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What five cofacts are needed by pyruvate dehydrogenase complex?
Thiamine, Riboflavin, Niacin, Pantothenate, & Lipoic acid
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What are the two purely ketogenic amino acids?
Lysine & Leucine
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What can high fat content, Lysine & Leucine be used to treat?
Pyruvate dehydrogenase deficiency (congenital or acquired)
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What are the four irreversible enzymes for gluconeogenesis?
Pyruvate carboxylase, PEP carboxykinase, Fructose 1-6 bisphophatase, Glucose -6-phosphatase
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Rotenone, CN, antimycin A, CO are all?
Electron transport inhibitors
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What is an ATPase inhibitor?
Oligomycin
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2,4 DNP and aspirin are both?
uncoupling agents of the electron transport chain
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What creates a osmotic pressure that causes water to enter the cell and produce osmotic damage?
Sorbitol, made from glucose via Aldose reductase
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What can be given to decrease ammonia levels?
Benzoate or phenylbutyrate
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A patient who presents with tremor, slurring of speech, somnolence, vomiting, cerebral edema, and blurring of vision is suffering an intoxication of?
Ammonia
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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
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