Deevska Review Flashcards

1
Q

Why is vitamin C (ascorbic acid) important for collagen

A

Required as a cofactors for the enzymes involved in hydroxylation of proline and lysine residues in collagen

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2
Q

What does a deficiency in vit C do?

A

Lack of proline and lysine hydroxylation lead to impairment of the interchain H bond formation which prevents the formation of a stable triple helix and affects proper cross-linking

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3
Q

What is the effect of vitamin C deficiency on collagen directly

A

Greatly decrease the tensile strength of the assembled fiber

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4
Q

What disease is caused by vitamin c deficient and what are the symptoms

A

Scurvy

  • easy bruising
  • loose teeth an bleeding gums
  • poor wound healing
  • poor bone development
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5
Q

What is the main difference between the structure of elastin and collagen?

A

Collagen contains hydroxyproline and hydroxylysine whereas elastin contains scant amounts of this

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6
Q

Extensively interconnected, rubbery network that can stretch and bend in any direction when stressed, giving connective tissue elasticity

A

Elastin

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7
Q

What is the makeup of elastin

A
  • insoluble protein polymer synthesized from a precursor tropoelastin
  • rich in proline and lysine but contains scant amounts od hydroxyproline and hydroxylysine
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8
Q

_________ modifies lysyl side chains in tropoelastin forming a desmosine corsslinking which produces ______

A

Elastin

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9
Q

What AA are both elastin and collagen abundant in

A

Proline and glycine

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10
Q

Fibrous proline composed of alpha chains forming triple stranded helix

A

Collagen

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11
Q

What does collagen have that elastin does not have much of

A

Contains hydroxyproline and hydroxylysine formed by post translational hydroxylation of proline and lysin residues

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12
Q

Collage requires what as a reducing agent

A

O2, Fe2+, and vit C

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13
Q

What is the most abundant protein in the human body

A

Collagen

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14
Q

What types of collagen in cornea

A

I and VIII

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15
Q

What types of collagen in vitreous

A

II

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16
Q

Large complexes of heteropolysaccharide chains associated with a small amount of protein
95% carbohydrates
5% protein

A

Proteoglycans

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17
Q

Protein with a variable but typically small amount of carbohydrate

A

Glycoproteins

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18
Q

Produce a gel like matrix, form basis of the body’s ground substance, function to form ECM, serves as flexible support for ECM, sieve influencing the movement of materials through the ECM, contributes to the viscous lubricating properties of mucous secretions

A

Proteoglycans

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19
Q

What are proteoglycan aggregates composed of?

A

1 proteoglycan monomers

  • GAGs
  • a core protein
    2. Hyaluronic acids
    3. Link protein
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20
Q

What is the anchor point for the proteoglycan

A

Hyaluronic acid

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21
Q

Small protein that stabilizes the association between the core protein and hyaluronic acid in a proteoglycan

A

Link protein

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22
Q

Part of the proteoglycan monomer that us linked to a serine residue with 100s of monosaccharide chain extending out from it

A

Core protein

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23
Q

Long unbranched, heteropolysaccharide chains, six different types, always found external to the cytosol in the proteoglycan

A

GAGs

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24
Q

Characteristics of GAGs

A
  • unbranched
  • repeating disaccharide units

Large complexes of NEGATIVELY charged heteropolysaccharide chains

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25
Q

What gives the GAGs their unique function

A

Negative charge

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26
Q

What kind of charge does a GAG have and why

A

Negative because carboxyl groups and sulfate groups

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27
Q

What does the negative charge on the GAGs contribute to

A
  • hydraulic absorption and resilience of synovial fluid and vitreous humor of the eye
  • lubricating effects of mucous and synovial fluid
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28
Q

Functions of membrane bound glycoproteins

A
  • cell surface recognition by other cells, hormones, viruses
  • cell surface antigenicity
  • formation of ECM and mucins
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29
Q

non membrane bound glycoproteins

A
  • almost all globular proteins secreted in the plasma are glycoproteins
  • many of the lysosomal proteins are glycoproteins
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30
Q

Structure of the carbohydrate chain of a glycoprotein

A
  • short
  • no serial repeats
  • often branched
  • may/may not be negatively charged
  • highly variable in amount and comp of carbohydrate
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31
Q

Structure of the linkage between carbohydrate and protein in a glycoproteins

A
  • N-glycosidic link: sugar chain attached to abide group of asparagine
  • O-glycosidic link: sugar chain is attached to the hydroxyl group of either serine or threonine

Glycoprotein can contain one of these or both of these

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32
Q

What are the 6 major classes of GAGs

A
  • chondroitin sulfate
  • keratin sulfate I and II
  • hyaluronic acid
  • dermatan sulfate
  • heparin
  • heparin sulfate
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33
Q

What is the most abundant GAG in the body?

A

Chondroitin sulfate

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34
Q

Where is chondroitin sulfate (GAGs) found

A

Cartilage, tendones, ligaments, and aorta

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35
Q

Where is keratin sulfate I and II found

A

KSI: in coreans
KSII: loose connective tissue proteoglycan aggregates with chondroitin sulfate

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36
Q

What is the only GAG not attached to a core protein?

A

Hyaluronic acid

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37
Q

What is the only GAG not sulfated?

A

Hyaluronic acid

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38
Q

Where is hyaluronic acid found

A
  • animal and bacteria

- synovial fluid in joints, vitreous, umbilical cord, loose connective tissue, cartilage

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39
Q

Where is dermatan sulfate found

A

Skin blood vessels, and heart valves

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40
Q

What is the only GAG that is intercellular

A

Heparin

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41
Q

Where is heparin found and what does it do

A

Intracellular component of mast cells that line arteries, especially in liver, lungs, and skin

Serves as an anticoagulant

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42
Q

Where is heparin sulfate found

A

Basement membrane

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43
Q

What is the difference between heparan and heparin sulfate?

A

Heparin sulfate-some glucosamine are acetylated and there are fewer sulfate groups

44
Q

What is the most heterogenous GAG

A

Keratin sulfate

45
Q

A defect in the sulfation of the growing glysoaminoglycan chains results in one of several disorders that affect the proper development and maintainence of the skeletal system

A

Chondrodystrophy

46
Q

What kind of inherited disorder is chondrodystrophy

A

Autosomal recessive

47
Q

Characteristics of chondrodystrophy

A
  • dwarfism
  • over 100 specific skeletal dysplasias
  • rare
48
Q

Progressive disorders characterized by glycosaminoglycans accumulating in the lysosomes of various tissues results in oligosaccharides in the urine

A

Mucopolysaccharides

49
Q

What kind of inheritance is mucopolysaccharidoses

A

Autosomal recessive

50
Q

Diagnosis of mucopolysaccharidoses

A
  • the specific MPS is determined by identifying the structure present at the non-reducing end of the oligosaccharide
  • diagnosis is confirmed by measuring the patients cellular level of lysosomal hydolases
51
Q

Symptoms of mucopolysaccharidoses

A
  • apparently normal at birth, gradually deteriorate, could cause childhood mortality
  • skeletal and extracellular matric deformities and mental retardation
52
Q

What are all the MPS diseases

A
  • hurler
  • hunter
  • sanfilippo
  • sly
53
Q

Ocular symptoms of hurler syndrome (MPS)

A

Corneal clouding

54
Q

Ocular symptoms of hunter syndrome (MPS)

A

None

55
Q

Ocular symptoms of sanfillipo syndrome

A

None

56
Q

Ocular symptoms of sly syndrome (MPS)

A

Corneal clouding

57
Q

Which 2 MPS cause corneal clouding

A

Hurler and Sly

58
Q

Deficiency in the ability to phosphorylate mannose.

A

I-cell disease

59
Q

What kind of disease is I-cell

A

Lysosomal storage disease. Accumulation of large lysosomal inclusion bodies composed of the missing hydrolase substrate

60
Q

Symptoms of I cell

A
  • skeletal abnormalities, restricted joint movement, coats dysmorphic facial features, severe psychomotor impairment
  • death likely prior to 8 years old
61
Q

What is the genetic predisposition for DM type I

A

Moderate

62
Q

What is the genetic predisposition for DM type II

A

Very strong

63
Q

What biochemical and physiological changes is often seen in type I but not type II

A

Ketosis

64
Q

Which type of DM has no insulin

A

Type I

65
Q

What type of diabetes has insulin, but has a resistance

A

Type II

66
Q

Metabolic changes in type I DM

A

Hyperglycemia
Ketosis and ketoacidosis
Hyperlipidemia

67
Q

Symptoms of type I DM

A
  • Polyuria, polydipsia, and polyphagia
  • ketoacidosis
  • fatigue
  • weight loss
68
Q

How does the body react in type I DM

A

As if it is in starvation state

69
Q

What is type II Dm a result of

A
  • insulin resistance

- dysfunctional beta cells

70
Q

The decreased ability of target tissues, such as liver, adipose, and muscle, to respond properly to normal (or elevated) circulating concentrations of insulin

A

Insulin resistance

71
Q

Why does insulin resistance alone not type II DM?

A

Type II DM develops in insulin resistance individuals who also show impaired B cell function that cannot longer secrete sufficient insulin to compensate for elevated blood glucose levels

72
Q

What is the leading cause of adult blindness?

A

Retinopathy

  • an abnormal metabolic change from diabetes
  • in tissues in which glucos uptake is not dependent on insulin, hyperglycemia causes an increase in uptake
73
Q

What is BMI

A

Body mass index

  • provides indirect measure of body fat
  • moderately correlated with more direct measures of body fat obtained from other more direct methods
  • NOT a diagnostic of the body fatness or the health of an individual
74
Q

What is normal BMI

A

18.5-<25

75
Q

Waist to hip ratio

A
  • Reflects central abdominal fat amount

- excess central fat correlates with higher risk for morbidity and mortality

76
Q

What is a better measurement for obesity, BMI or waist to hip ratio?

A

Waist to hip ratio

77
Q

Why is hip to waist ratio a better tool for assessing obesit?

A

Visceral fat ( around the middle) is linked with metabolic syndrome

78
Q

What was the first identified adipocytes peptide hormone

A

Lepton

79
Q

What kind of signal does lepton give

A

Anorexigenic signal (loss of appetite)

80
Q

Where does lepton get secreted

A

From white adipose tissue

81
Q

What recognized leptin

A

Leptin receptors in the hypothalamus

Highest levels in evening hours

Secreted in proportion to the size of fat stores

82
Q

“Not all fats are the same”

A

Data not show that the type of fat is a more important risk factor for disease than the total amount of fat consumed

83
Q

Which fats are the best?

A

Monounsaturated fats

84
Q

What are bad fats

A

Trans fats
Some saturated fats
PUFA to some degree

85
Q

A wasting disorder characterized by loss of appetite and muscle atrophy

A

Cachexia

86
Q

Protein deprivation is relatively greater than the reduction in total calories

A

Kwashiorkor

Fatty liver and edema

87
Q

Calorie deprivation is relatively greater than the reduction in proline

A

Marasmus

No edema

88
Q

Protein energy malnutrition

A
  • decreases appetite
  • alter how nutrients are digested or absorbed
  • inadequate intake of protein and/or energy is the primary cause
  • depressed immune system
  • death from secondary infection is common
89
Q

What are the fat soluble vitamins

A

DEAK

90
Q

Usually precursors of coenzymes for different enzymes

A

Water soluble vitamins

91
Q

What is the only fat soluble vitamin that functions as coenzyme

A

Vitamin K

92
Q

Where is vitamin A stored?

A

Liver and adipose tissue

93
Q

What is another name for vitamin A

A

Retinoids

94
Q

Deficiencies of vitamin A

A
  • night blindness

- severe: xerophthalmia

95
Q

What is a component of rhodopsin

A

Vit A

96
Q

Hypervitaminosis of vitamin A

A
  • dry skin
  • cirrhotic liver
  • mimic brain tumor
97
Q

Where is vitamin D stored

A

Plasma

98
Q

What is the active form of vitamin D

A

Calcitrol

99
Q

Precursors of active form of vit D

A

D2 and D3

100
Q

Functions of vit D

A

Maintain adequate plasma levels of calcium

101
Q

Deficicneis in vit D

A

-demineralization of bone resulting in rickets in children and osteomalacia in adults

102
Q

Hyperviatminosis of vit D

A

-hypercalcemia-lead to deposition of calcium in many organs, particularly the arteries and kidneys

103
Q

What are the different forms of vit K

A

K1 in plants

K2 produced by intestinal bacteria

104
Q

Functions of vit K

A

Posttranslational modification of a number of proteins involved with blood clotting

105
Q

Deficiencies of vit K

A
  • unusual because intestinal bacteria usually produce sufficient amounts
  • in condition associated with loss of bacteria functions
  • newborns receive single dose
106
Q

Hypervitaminosis of vit K

A

Hemolytic anemia and jaundice in the infants in prolonged use