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