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AAA Biochemistry > Special Topics > Flashcards

Special Topics Flashcards

1
Q

GAGs are made of alternating

A 
Acidic sugar (glucuronic acid/iduronic acid)
Amino sugar (n-acetylglucosamine/galactosamine)
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2
Q

All GAG are covalently attached to proteins to form proteoglycans except

A

Hyaluronic acid

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3
Q

GAG linked to proteins via?

A

Trihexoside serine-o-glycosidic bonds

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4
Q

GAGs

With structural role in sclera

A

Dermatan sulfate

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5
Q

GAGs

Role in corneal transparency

A

Keratan sulfate and dermatan sulfate

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7
Q 
Glycoproteins:
Major classes (3)
A

O linked
N linked
GPI linked

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8
Q

Glycoproteins:
Bound to serine and threonine
Golgi
Hydroxyl group

A

O-linked

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9
Q

Glycoproteins:
Bound to asparagine
Amide group
ER

A

N-linked

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10
Q

Glycoproteins:

O-linked in fertilization

A

ZP3

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11
Q

Deficient phosphorylation of mannose residues in N-linked glycoprotein enzymes –> no labelling of pre-enzymes so that they can be transported to lysosomes
Presents with coarse facial features, skeletal abnormalities, severe psychomotor impairment

A

I-cell disease

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12
Q

Glycoproteins:

In influenza virus, these allows new viruses to exit the cell

A

Neuraminidase

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13
Q

Glycoproteins:
HIV type 1:
Used to attach to cell?
Used to fuse with the host?

A

GP 120

GP 41

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14
Q

Glycoproteins:

Disease associated with an altercation in the glycosylation of IgG; no galactose in Fc; ends in GlcNAc; IgM attacks IgG

A

Rheumatoid arthritis

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15
Q

Attachment is mediated by a GPI present on the surface of its parasite

A

Malaria

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16
Q

GAG found in synovial fluid, vitreous humor, and loose connective tissue

A

Hyaluronic acid

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17
Q

Sugars in Hyaluronic acid

A

GlcNAc, GlcUA

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18
Q

GAG found in cartilage, bone and cornea

A

Chondroitin sulfate

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19
Q

Sugars found in Chondroitin sulfate

A

GalNAc, GlcUA

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20
Q

GAG in cornea and loose connective tissue

A

Keratan sulfate

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21
Q

Sugars in Keratan sulfate

A

GlcNAc, Gal

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22
Q

GAG in mast cells

A

Heparin

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23
Q

Sugars in Heparin

A

GlcN, IduA

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24
Q

GAG in skin and aortic wall

A

Heparan sulfate

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25
Q

Sugars in Heparan sulfate

A

GlcN, GlcUA

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26
Q

GAG found in wide distribution

A

Dermatan sulfate

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27
Q

Sugars in dermatan sulfate

A

GalNAc, IdUA

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28
Q

MPS Disease:
Deficiency and findings:

Alpha l iduronase
Corneal clouding

A

Type I H

Hurler’s

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29
Q

MPS Disease:
Deficiency and findings:

Alpha l iduronase

Cardiomyopathy
Mental retardation

A

Type I S

Scheie’s

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30
Q

MPS Disease:
Findings:

Spasticity then loss of motor function
Mental retardation
Hyperactivity

A

Type III

San Filippo syndrome

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31
Q

4 enzymes in San filippo syndrome?

A

Heparan sulfamidase
N-acetylglucosaminidase
N-acetylglucosamine sulfatase
N-acetylglucosamine

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32
Q

MPS Disease:
Deficiency and findings:

Iduronate sulfatase

NO corneal clouding
Cardiomyopathy
Mental retardation

A

Type II

Hunter syndrome

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33
Q

MPS Disease:
Deficiency and findings:

Galactose 6 sulfatase

No CNS involevement
Skeletal dysplasia and short stature

A

Type IV

Morquio’s syndrome

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34
Q

MPS Disease:
Deficiency and findings:

B-glucuronidase

Corneal clouding
Hepatomegaly
Skeletal dysplasia and short stature
Mental retardation

A

Type VII

Sly syndrome

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35
Q

Glycoprotein vs proteoglycan

Which is longer, linear and with repeating sugar units?

A

Proteoglycans

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36
Q

Glycoprotein vs proteoglycan

Which is shorter, branched, with no repeating sugar?

A

Glycoprotein

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37
Q

Average daily nutrient intake level estimated to meet the requirement of 50% of healthy individuals in a particular life stage and gender group

A

Estimated average requirement

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38
Q

Average daily dietary intake level that is sufficient to meet the requirements of 95% of all individuals in a life stage and gender group

A

Recommended dietary allowance

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39
Q

Set in the absence of scientific evidence to compute for EAR or RDA

A

Adequate intake

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40
Q

Highest average nutrient intake level to pose no risk of adverse health effects

A

Tolerated upper intake level

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41
Q

Estimated energy requirement:
Sedentary?
Moderately active?
Very active?

A

30-35-40 kcal/kg/day

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42
Q

Energy requirement:
Fat?
CHO?
Protein?

A

Energy requirement:
Fat? 20-35%
CHO? 45-65%
Protein? 10-35%

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43
Q

Energy content:
Fats?
CHO/Proteins/Ketones?
Alcohol?

A

Energy content:
Fats? 9 kcal/g
CHO/Proteins/Ketones? 4 kcal/g
Alcohol? 7 kcal/g

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44
Q

How is energy used?
BMR?
Diet induced thermogenesis?
Exercise?

A

How energy is used?
BMR? 60%
Diet induced thermogenesis? 10%
Exercise? 30%

45
Q 
Kwashiorkor vs Marasmus
>1 year old
Stunting
With edema
Very low plasma protein
With fatty liver, skin lesions and depigmented hair
A

Kwashiorkor

46
Q 
Kwashiorkor vs Marasmus
<1 year old
Stunting and extreme wasting
Without edema
Normal plasma protein
With anemia
A

Marasmus

47
Q

Vitamin A

3 forms?

A

Retinoic acid
Retinol
Retinol

48
Q 
Vitamin A
Functions?
Retinoic acid
Retinol
Retinol
A

Retinoic acid - epithelial growth regulators
Retinol - gametogenesis in gonads
Retinol - rod and cone cells for vision

49
Q

Vitamin A
Storage form?
Transport form?

A

Vitamin A
Storage form? Retinyl ester
Transport form? Retinol binding protein

50
Q

Vitamin D

3 forms

A

Ergocalciferol (D2) - plant, milk
Cholecalciferol (D3) -animal, skin
Calcitriol

51
Q

Vitamin D
Precursor?
Storage form?
Transport form?

A

Vitamin D
Precursor? 7 dehydrocholesterol
Storage form? 25 OH D3
Transport form? 1,25 OH D3

52
Q

PTH vs Vitamin D:
Increases PO4
Increases intestinal absorption of calcium

A

Vitamin D

53
Q

PTH vs Vitamin D:
Decreases PO4
Indirectly Increases intestinal absorption of calcium

A

PTH

54
Q

Vitamin Deficiency in:
RBC fragility
Neurologic dysfunction

A

Vitamin E

55
Q 
Vitamin toxicity in:
Hyperkeratosis
Hepatomegaly
Pseudotumor cerebri
Fractures
Teratogenicity
A

Vitamin A

56
Q

Vitamin K

3 forms

A

Phylloquinone (K1)
Menaquinone (K2)
Menadione (Synthetic)

57
Q

Vitamin K toxicity?

A

Jaundice + hemolytic anemia

58
Q

Vitamin K deficiency?

A

Rare

Hemorrhagic disease of the newborn

59
Q

Water soluble vitamins generally nontoxic except:

A

B6

B12

60
Q

Glycosphingolipids in ABO system?

A

A: N-acetylgalactosamine
B: Galactose
AB:Both

61
Q

Cofactor in:
Proline and lysine hydroxylation (collagen synthesis)
Dopamine B hydroxyoase (dopa to norepi)
Fe3+ to Fe2+

A

Vit C

62
Q

Biotin Carboxylation reactions

A

Pyruvate - gluconeogenesis
Acetyl coa - FA synthase
Propionyl coa - FA

63
Q

Vitamin Def:
Avidin in eggwhites
Dermatitis
Enteritis

A

Biotin

64
Q

Vit Def:
Megaloboastic anemia with no neuro symptoms
Neural tube defects

A

Folic acid (B9)

65
Q

Folic acid (B9) supplementation in pregnant mothers

A

400 mcg daily

66
Q

Folic acid structure:

A

Pterin ring + PABA + glutamate residues

67
Q

Folic acid:

Active form:

A

THF

68
Q

Folic acid:
Enzymes:
PABA to DHF
DHF to THF

A

Dihydropteroate synthase

Dihydrofolate reductase

69
Q 
Vitamin functions as:
Coenzyme for 1-carbon transfer
Methylation reaction (purine and thymine synthesis)
A

Folic acid (B9)

70
Q

Vitamin B12 active forms:
For methionine synthesis?
For isomerization reactions?

A

5-deoxyadenosylcobalamin

Methylcobalamin

71
Q 
Vitamin deficiency in:
Pernicious anemia
Neuropsychiatric sx
Schillin test as Dx
Anti IF as Dx
A

Cobalamin

72
Q

Active form of B6

A

Pyridoxal phosphate

73
Q

Vitamin as coenzyme for:

AA transamination
Glycogen phosphorylase
Cystathione synthase
ALA synthase
Niacin synthesis from tryptophan
A

Pyridoxine (B6)

74
Q

Vit def in:

Isoniazid toxicity

A

Pyridoxine

75
Q

Pyridoxine toxicity

A

2g/day

76
Q

Vitamin as constituent of Coenzyme A

Acyl transfer & FA synthase

A

Panthothenic acid

77
Q

Vit def sx:
Dermatitis
Enteritis
Alopecia

A

Panthothenate

78
Q

Riboflavin active forms

A

FMN

FAD

79
Q

Vitamin as cofactors in redox reactions as electron carrier

A

Riboflavin

80
Q 
Deficiency symptoms:
Stomatitis
Cheilosis
Dermatitis
Corneal revascularization
A

Riboflavin

81
Q

Niacin active forms

A

NAD

NADP

82
Q

Vitamin as coenzymes in redox reactions, as electron carriers

A

Niacin

83
Q

Derived from tryptophan using B6

A

Niacin

84
Q

Niacin deficiency

A 
Pellagra
Hartnup (dec tryptophan absorption)
Carcinoid syndrome (inc tryptophan metabolism)
Isoniazid use (dec vit b6)
85
Q

Cofactor in pyruvate, alpha ketoglutarate and branched chain AA dehydrogenase
Cofactor in transketolase reactions in the HMP shunt

A

Thiamine

86
Q

Dry vs wet beriberi:

No heart failure, polyneuritis, symmetrical muscle wasting

A

Dry

87
Q

Wet beriberi

A

High output cardiac failure

88
Q

Grams of iron in the body

A

3-4 g

89
Q

2/3 of iron in:

A

Hemoglobin

90
Q

Grams of zinc

A

1.5 to 2.5 g

91
Q 
Constituent of:
Carbonic anhydrase
Cytoplasmic superoxide dismutase
Alcohol dehydrogenase
Carboxypeptidases
DNA and RNA polymerase
A

Zinc!

92
Q

Diet: meats, nuts, beans, wheat germ

A

Zinc!

93
Q

Dermatitis, diarrhea, alopecia secondary to zinc malabsorption

A

Acrodermatitis enteropathica

94
Q

Grams of copper

A

80-110 g

95
Q 
Enzyme cofactor:
Cytochrome oxidase
Dopamine b hydroxylase
Monoamine oxidase
Tyrosinase
Delta 9 desaturase
Lysyl oxidase
Cytoplasmic superoxide dismutase
A

COPPER!

96
Q 
Microcytic hypochromic anemia
Leukopenia
Hemorrhagic vascular changes
Bone demineralization
Hypercholesterolemia
Neurologic problems
A

COPPER!

97
Q

TPN and infants

Vit def?

A

COPPER!

98
Q

X linked recessive
Deficiency of ATP dependent membrane transporter for copper
Growth retardation, mental deficiency, seizures, arterial aneurysm, bone demineralization, brittle hair

A

Menkes Syndrome

99
Q

Hepatolenticular degeneration

Biliary excretion of copper is blocked, with accumulation at liver and brain

A

Wilson Disease

100
Q

Found in Wilson disease

A

Kayser fleischer rings

101
Q

Tx for Wilson disease

A

D-penicillamine

102
Q

Trace element which causes madness (psychosis, parkinsonism)

A

Manganese

103
Q

Occurs in few oxidase enzymes like xanthine oxidase

A

Molybdenum

104
Q

Def causes cardiomyopathy (endemic in china)

A

Selenium (Kashan disease)

105
Q

Trace element in glutathione peroxidase

A

Selenium

106
Q

Xenobiotics

Phase 1

A

Hydroxylation

107
Q

Xenobiotics

Phase 2

A

Conjugation

AAA Biochemistry (7 decks)

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