Flashcards in Carbohydrates Deck (39):
What are examples of anabolic reactions?
Protein and triglyceride synthesis, glycogenesis
What are example of catabolic reactions?
glycolysis, beta-oxidation, glycogenolysis
What are example amphibolic pathways?
Citric acid cycle
Oxidation of Glucose yields?
Reduction of glucose yields?
Ribose: Removal of hydroxyl group at C2 yields?
What is excreted in essential pentosuria?
Glucose + Fructose?
Glucose + Galactose?
Glucose + Glucose?
Bonds of Starch?
Bonds of cellulose?
What enzyme deficiency causes hemolytic anemia and is the most common enzyme defect in glycolysis?
What enzyme deficiency causes low exercise capacity especially on high carbohydrate diets?
What enzyme deficiency is the most common cause of congenital lactic acidosis; x linked dominant disease
TCA intermediates: delivers acetyl coA to cytosol for fatty acid synthesis via citrate shuttle
TCA intermediates: Used for heme synthesis and activation of ketone bodies in extrahepatic tissues
TCA intermediate: may be used for gluconeogenesis
What is the rate liming step of glycolysis?
What is the rate limiting step of TCA?
isocitrate -> alpha ketoglutarate (Isocitrate dehydrogenase)
What is the rate limiting step of Gluconeogenesis?
Fructose 1,6 bisphosphate -> Fructose 6-phosphate (Fructose 1,6-bisphosphatase)
What is the rate limiting step of glycogenesis?
Elongation of glycogen chains (creation of alpha 1,4 glycosidic bond by glycogen synthase)
What is the rate limiting step of glycogenolysis?7
Shortening of glycogen chains (Glycogen phosphorylase)
What is the coenzyme of glycogen phosphorylase in glycogenolysis?
What disease has glucose 6 phoshatase deficiency?
Von Gierke Disease
Clinical Features of Von Gierke Disease?
Increase glycogen in liver, hypoglycemia, lactic acidosis, ketosis, hyperlipidemia, hepatomegaly
What disease has deficiency in lysosomal acid maltase?
Clinical Features of Pompe Disease
Increase in glycogen in lysosomes; juvenile onset; hypotonia, death from heart failure by 2 years old; muscle dystrophy (adult onset)
What enzyme is deficient in Cori Disease?
What is deficient in McArdle syndrome?
What is an alternative pathway for oxidation of glucose in the liver?
Uronic Acid Pathway
An essential component of glycosaminoglycans and is required in detoxifixation reactions of insoluble compounds like bilirubin, steroids, morphine
Where does pentose phosphate pathway occur?
RBC and tissues that produce lipids (liver, adipose tissue, adrenals, thyroid, testes, lactating mamaries)
What is the rate limiting step of PPP?
Glucose 6 phosphate to 6 phosphogluconate (glucose 6 phosphate dehydrogenase)
What catalyzes reduced glutathione removing H2O2?
What disease involves decrease in NADPH in RBC and decreased activity of glutathione reductase causing free radicals and peroxidase accumulation?
What are the 2 pathologies of G6PD deficiency?
Heinz bodies and Bite cells
What disease is caused by deficiency in NADPH oxidase where molecular O2 is converted to superoxide in leukocytes?
Chronic Granulomatous Disease