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DEN 7101F - Human Structure and Function > Exam 1 9/11 Kingsley > Flashcards

Exam 1 9/11 Kingsley Flashcards

1
Q

What is in the ground substance of the ECM?

A

glycosaminoglycans (GAGs), proteoglycans, glycoproteins, fibers

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2
Q

Proteoglycans are ______ covered with ____

A

proteins; GAGs

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3
Q

What are GAGs structure/components?

A

Structure: long, unbranched polysaccharides of repeating sugars

Components: N-acetylgalactosamine, N-acetylglucosamine; with a uronic acid

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4
Q

GAGs are _____ charged

A

negatively

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5
Q

What groups on GAGs make them charged?

A

carboxyl groups, sulfates

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6
Q

GAGs attract _____ and _____

A

cations; water

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7
Q

What are the four main GAG groups?

A
  1. Hyaluron
  2. Chondroitin
  3. Heparin
  4. Keratan
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8
Q

Which of the four main GAG groups is not sulfated?

A

Hyaluron

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9
Q

Sulfated GAGs are usually attached to:

A

proteoglycans

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10
Q

Hyaluronic acid size

A

Extremely large (100-10,000 kDa)

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11
Q

Hyaluronic acid attracts a large volume of:

A

water

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12
Q

Hyaluronic acid can bind ______

A

proteoglycans

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13
Q

Proteoglycans exhibit a _____ _____ configuration

A

bottle brush

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14
Q

Proteoglycans components

A

core protein, linked sugars (GAGs)

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15
Q

True or false: syndecans are roughly similar sizes

A

false - variety of different sizes

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16
Q

How are syndecans different from proteoglycans?

A

they are transmembrane glycoproteins; E face (usually secreted)

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17
Q

What is the function of glycoproteins?

A

large macromolecules linking cell to the ECM

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18
Q

Most glycoproteins have what binding domains?

A

GAG/proteoglycan, collagen, integrin

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19
Q

_____ is most abundant in connective tissue

A

Fibronectin

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20
Q

Fibronectin is a ___ linked by ____ ____

A

dimer; disulfide bonds

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21
Q

What is the amino acid sequence for fibronectin integrin binding sequence?

A

RGD

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22
Q

Glycoprotein examples

A

Fibronectin, laminin, hemidesmosomes, dystroglycans

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23
Q

What type of mutation of RGD could cause elimination adhesion and binding affinity?

A

deletion, point mutation, frameshift mutation, etc. Anything that could cause a change in reading frame or the protein it codes for

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24
Q

Deletion or mutation of RGD reduces or eliminates:

A

adhesion and binding affinity

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25
Q

What is RGD?

A

Integrin binding domain on fibronectin. (common feature of integrin binding domains)

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26
Q

Where is laminin present?

A

Basal and external lamina

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27
Q

Laminin structure

A

Large heterotrimer that creates a “cross”

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28
Q

Laminin alpha chain binds:

A

neurites; LG domains bind carbohydrates and integrins

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29
Q

Laminin beta chain binds:

A

each unit binds sulfated lipids, together they bind type IV collagen

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30
Q

Laminin gamma chain binds:

A

collagen, sulfated lipids

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31
Q

Laminin-332 can bind to:

A

integrin, ground substance components

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32
Q

a6B4 is a component of:

A

component of hemidesmosomes

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33
Q

a6B4 is a _______

A

integrin (receptor for laminin 5)

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34
Q

What are dystroglycans?

A

glycoproteins with two subunits

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35
Q

Which subunit of dystroglycan is transmembrane?

A

b-dystroglycan

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36
Q

How does dystroglycan link with cytoskeleton?

A

binds dystrophin in the cytosol, dystrophin binds alpha-actinin, links with cytoskeleton (F-actin)

37
Q

Two major fibrous proteins

A

collagen and elastin

38
Q

Three major categories of collagen fibers

A
  1. fibril-forming
  2. fibril-associated
  3. network forming
39
Q

Collagen structure

A

tropocollagen helix/triple helix, glycine every 3rd aa, hydroxyproline, hydroxylysine

40
Q

Collagen synthesis steps

A
  1. translation in RER
  2. modified in cisternae
  3. signal sequence removed
  4. proline, lysine hydroxylated
  5. glycosylation of hydroxyproline and hydroxylisine
  6. forms procollagen helix
  7. modified again in golgi
  8. secreted via trans golgi
41
Q

Collagen secretion

A
  1. procollagen peptidase modifies procollagen helix
  2. forms tropocollagen
  3. tropocollagen self assembles to form collagen fibril
42
Q

Unique aspect tropocollagen structure

A

overlaps and gaps

43
Q

Which types of collagen are fibril-forming?

A

type 1, type 2, type 3, type 5

44
Q

Which type of collagen is not fibril forming?

A

Type 4

45
Q

Which disease is caused by deficiency/absence of Type 1 collagen?

A

osteogenesis imperfecta

46
Q

Osteogenesis imperfecta is also known as:

A

brittle bone disease

47
Q

What causes disruption of collagen helix in osteogenesis imperfecta?

A

Glycine is substituted with a bulkier amino acid, helix can’t form properly

48
Q

Which disease is caused by deficiency/absence of Type IV or III collagen?

A

Dentinogenesis imperfecta

49
Q

_____ _____ can occur in rare forms of osteogenesis imperfecta

A

dentinogenesis imperfecta

50
Q

Dentinogenesis imperfecta symptoms

A

discolored teeth (blue-grey, yellow-brown); translucent teeth; weak, easily fall out or fracture

51
Q

Which disease is caused by deficiency/absence of Type V (classic) or III (vascular) collagen?

A

Ehlers Danlos Syndrome

52
Q

Ehlers Danlos Syndrome symptoms

A

Classic (Type V) joint hypermobility and hyperextensible skin
Vascular (Type III) problem with artery ruptures (aneurysms, often lethal

53
Q

Collagen has high _____ ____

A

tensile strength

54
Q

_____ _____ provide flexibility

A

Elastin fibers

55
Q

Which amino acids are found in elastin?

A

glycine, lysine, alanine, valine, proline

56
Q

True or false: like collagen, elastin also contains hydroxylysine

A

False

57
Q

In elastin, lysine forms:

A

four elastin proteins bond and desmosine cross links

58
Q

_____ is caused by chromosome 7 partial deletion

A

Williams-Beuren syndrome

59
Q

Loss of elastin can cause:

A

supravalvular aortic stenosis

60
Q

What symptom is among clinical features of Williams Beuren syndrome?

A

Esotropia - form of strabismus

61
Q

What is observed regarding gene expression of Williams Beuren syndrome

A

Partial deletion of chromosome 7 which codes for elastin, so elastin cannot be produced

62
Q

Basement membrane components:

A
  1. basal lamina
  2. lamina lucida
  3. lamina densa
  4. lamina reticularis
63
Q

Components of lamina densa (basement membrane)

A

GAGs, proteoglycans, glycoproteins

64
Q

True or false: basement membrane has very specific architecture/organization

A

True

65
Q

Basal lamina functions

A
  1. flexible, firm support for overlying epithelium
  2. establishing cell polarity
  3. facilitating tissue differentiation
  4. molecular filter
66
Q

Which diseases are caused by auto-antibodies?

A
  1. Epidermolysis bullosa acquisita
  2. pemphigus
  3. Pemphigoid
  4. Goodpasture syndrome
  5. Linear IgA disease
67
Q

Epidermolysis bullosa acquisita (EBA) caused by:

A

Auto-immunity IgG develops against Type VII procollagen; leads to dermal-epidermal separation

68
Q

True or false: Epidermolysis bullosa acquisita (EBA) is hereditary

A

False - most epidermolysis bullosa is hereditary, but EBA is not

69
Q

Epidermolysis bullosa acquisita (EBA) is described as a ______ ______ disease

A

chronic sub-epidermal

70
Q

What do Type VII collagen fibers do?

A

anchoring fibrils in basement membrane

71
Q

Epidermolysis bullosa acquisita (EBA) is characterized by what symptoms?

A

spontaneous or trauma-induced blisters in areas of high contact (feet, hands, mouth); acantholysis

72
Q

Pemphigus and pemphigoid are groups of:

A

rare autoimmune blistering diseases

73
Q

What causes pemphigus?

A

auto=antibodies IgG form against desmoglein-3 of desmosomes

74
Q

Pemphigus mostly affects:

A

desmosomes of stratified squamous epithelium

75
Q

Pemphigoid is caused by:

A

auto-antibodies of IgG to BP-1 (hemidesmosome) antigen

76
Q

Pemphigoid is similar in appearance to:

A

pemphigus except WITHOUT acantholysis

77
Q

Pemphigoid is ______

A

cicatrial (mucous membrane) - oral

78
Q

What kind of disease is goodpasture syndrome?

A

anti-glomerular basement membrane disease

79
Q

What causes goodpasture syndrome?

A

autoantibodies to alpha-3 subunit of Type IV collagen (antibodies against basement membrane in lungs and kidneys)

80
Q

Goodpasture syndrome may lead to:

A

permanent lung/kidney damage of death

81
Q

How is goodpasture syndrome treated?

A

with immunosuppressants

82
Q

True or false: goodpasture syndrome is a common disease

A

false - exceedingly rare

83
Q

What triggers goodpasture syndrome?

A

lung insults such as smoke, cocaine inhalation, metal dust, organic solvents

84
Q

Linear IgA (bullous) dermatitis is a _____ ___ disease

A

sub-epidermal bullous

85
Q

Which diseases are characterized as sub-epidermal?

A

Linear IgA, Epidermolysis bullous acquisita, possibly pemphigus (may form bullous)

86
Q

What causes Linear IgA disease?

A

Linear IgA deposition in basement membrane zone (BMZ); results in loss of adhesion at dermal-epidermal junction, blisters. may cause vesicles, ulcers

87
Q

In Linear IgA, antibody deposition activates:

A

complement, neutrophils

88
Q

True or false: there is only one antigenic target identified for Linear IgA disease

A

False - different targets (lamina lucida, lamina densa)

DEN 7101F - Human Structure and Function (23 decks)

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