Carbohydrates

Question 1

What are examples of anabolic reactions?

Answer 1

Protein and triglyceride synthesis, glycogenesis

Question 2

What are example of catabolic reactions?

Answer 2

glycolysis, beta-oxidation, glycogenolysis

Question 3

What are example amphibolic pathways?

Answer 3

Citric acid cycle

Question 4

Oxidation of Glucose yields?

Answer 4

Glucoronic acid

Question 5

Reduction of glucose yields?

Answer 5

Sorbitol

Question 6

Ribose: Removal of hydroxyl group at C2 yields?

Answer 6

deoxyribose

Question 7

What is excreted in essential pentosuria?

Answer 7

Xylulose

Question 8

Glucose + Fructose?

Answer 8

Sucrose

Question 9

Glucose + Galactose?

Answer 9

Lactose

Question 10

Glucose + Glucose?

Answer 10

Lactose

Question 11

Bonds of Starch?

Answer 11

alpha-glycosidic

Question 12

Bonds of cellulose?

Answer 12

beta-glycosidic

Question 13

What enzyme deficiency causes hemolytic anemia and is the most common enzyme defect in glycolysis?

Answer 13

Pyruvate kinase

Question 14

What enzyme deficiency causes low exercise capacity especially on high carbohydrate diets?

Answer 14

Muscle phosphofructokinase

Question 15

What enzyme deficiency is the most common cause of congenital lactic acidosis; x linked dominant disease

Answer 15

Pyruvate dehydrogenase

Question 16

TCA intermediates: delivers acetyl coA to cytosol for fatty acid synthesis via citrate shuttle

Answer 16

Citrate

Question 17

TCA intermediates: Used for heme synthesis and activation of ketone bodies in extrahepatic tissues

Answer 17

succinyl coA

Question 18

TCA intermediate: may be used for gluconeogenesis

Answer 18

Malate

Question 19

What is the rate liming step of glycolysis?

Answer 19

phosphofructokinase-1

Question 20

What is the rate limiting step of TCA?

Answer 20

isocitrate -> alpha ketoglutarate (Isocitrate dehydrogenase)

Question 21

What is the rate limiting step of Gluconeogenesis?

Answer 21

Fructose 1,6 bisphosphate -> Fructose 6-phosphate (Fructose 1,6-bisphosphatase)

Question 22

What is the rate limiting step of glycogenesis?

Answer 22

Elongation of glycogen chains (creation of alpha 1,4 glycosidic bond by glycogen synthase)

Question 23

What is the rate limiting step of glycogenolysis?7

Answer 23

Shortening of glycogen chains (Glycogen phosphorylase)

Question 24

What is the coenzyme of glycogen phosphorylase in glycogenolysis?

Answer 24

Pyridoxal phosphate

Question 25

What disease has glucose 6 phoshatase deficiency?

Answer 25

Von Gierke Disease

Question 26

Clinical Features of Von Gierke Disease?

Answer 26

Increase glycogen in liver, hypoglycemia, lactic acidosis, ketosis, hyperlipidemia, hepatomegaly

Question 27

What disease has deficiency in lysosomal acid maltase?

Answer 27

Pompe Disease

Question 28

Clinical Features of Pompe Disease

Answer 28

Increase in glycogen in lysosomes; juvenile onset; hypotonia, death from heart failure by 2 years old; muscle dystrophy (adult onset)

Question 29

What enzyme is deficient in Cori Disease?

Answer 29

Debranching enzyme

Question 30

What is deficient in McArdle syndrome?

Answer 30

Muscle phosphorylase

Question 31

What is an alternative pathway for oxidation of glucose in the liver?

Answer 31

Uronic Acid Pathway

Question 32

An essential component of glycosaminoglycans and is required in detoxifixation reactions of insoluble compounds like bilirubin, steroids, morphine

Answer 32

Glucoronic acid

Question 33

Where does pentose phosphate pathway occur?

Answer 33

RBC and tissues that produce lipids (liver, adipose tissue, adrenals, thyroid, testes, lactating mamaries)

Question 34

What is the rate limiting step of PPP?

Answer 34

Glucose 6 phosphate to 6 phosphogluconate (glucose 6 phosphate dehydrogenase)

Question 35

What catalyzes reduced glutathione removing H2O2?

Answer 35

Glutathione Peroxidase

Question 36

What disease involves decrease in NADPH in RBC and decreased activity of glutathione reductase causing free radicals and peroxidase accumulation?

Answer 36

G6PD deficiency

Question 37

What are the 2 pathologies of G6PD deficiency?

Answer 37

Heinz bodies and Bite cells

Question 38

What disease is caused by deficiency in NADPH oxidase where molecular O2 is converted to superoxide in leukocytes?

Answer 38

Chronic Granulomatous Disease

Question 39

In chronic granulomatous disease, which bacteria is responsible?

Answer 39

catalase positive bacteria