Carbohydrates Flashcards

1
Q

What are examples of anabolic reactions?

A

Protein and triglyceride synthesis, glycogenesis

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2
Q

What are example of catabolic reactions?

A

glycolysis, beta-oxidation, glycogenolysis

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3
Q

What are example amphibolic pathways?

A

Citric acid cycle

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4
Q

Oxidation of Glucose yields?

A

Glucoronic acid

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5
Q

Reduction of glucose yields?

A

Sorbitol

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6
Q

Ribose: Removal of hydroxyl group at C2 yields?

A

deoxyribose

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7
Q

What is excreted in essential pentosuria?

A

Xylulose

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8
Q

Glucose + Fructose?

A

Sucrose

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9
Q

Glucose + Galactose?

A

Lactose

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10
Q

Glucose + Glucose?

A

Lactose

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11
Q

Bonds of Starch?

A

alpha-glycosidic

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12
Q

Bonds of cellulose?

A

beta-glycosidic

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13
Q

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

A

Pyruvate kinase

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14
Q

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

A

Muscle phosphofructokinase

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15
Q

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

A

Pyruvate dehydrogenase

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16
Q

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

A

Citrate

17
Q

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

A

succinyl coA

18
Q

TCA intermediate: may be used for gluconeogenesis

A

Malate

19
Q

What is the rate liming step of glycolysis?

A

phosphofructokinase-1

20
Q

What is the rate limiting step of TCA?

A

isocitrate -> alpha ketoglutarate (Isocitrate dehydrogenase)

21
Q

What is the rate limiting step of Gluconeogenesis?

A

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

22
Q

What is the rate limiting step of glycogenesis?

A

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

23
Q

What is the rate limiting step of glycogenolysis?7

A

Shortening of glycogen chains (Glycogen phosphorylase)

24
Q

What is the coenzyme of glycogen phosphorylase in glycogenolysis?

A

Pyridoxal phosphate

25
Q

What disease has glucose 6 phoshatase deficiency?

A

Von Gierke Disease

26
Q

Clinical Features of Von Gierke Disease?

A

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

27
Q

What disease has deficiency in lysosomal acid maltase?

A

Pompe Disease

28
Q

Clinical Features of Pompe Disease

A

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

29
Q

What enzyme is deficient in Cori Disease?

A

Debranching enzyme

30
Q

What is deficient in McArdle syndrome?

A

Muscle phosphorylase

31
Q

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

A

Uronic Acid Pathway

32
Q

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

A

Glucoronic acid

33
Q

Where does pentose phosphate pathway occur?

A

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

34
Q

What is the rate limiting step of PPP?

A

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

35
Q

What catalyzes reduced glutathione removing H2O2?

A

Glutathione Peroxidase

36
Q

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

A

G6PD deficiency

37
Q

What are the 2 pathologies of G6PD deficiency?

A

Heinz bodies and Bite cells

38
Q

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

A

Chronic Granulomatous Disease

39
Q

In chronic granulomatous disease, which bacteria is responsible?

A

catalase positive bacteria