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MK ACVD Exam > BMZ and adhesion molecules > Flashcards

BMZ and adhesion molecules Flashcards

1
Q

What is the keratin composition of intermediate filaments?

A

Keratin-5 (basic), keratin-14 (acidic)

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2
Q

Define intermediate filament

A

Connection between the keratinocyte nucleus to hemidesmosomes on the keratinocyte membrane. Responsible for structure of basal keratinocytes

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3
Q

What is the composition of the inner plaque of the hemidesmosome?

A

Plectin, BPAG1-e

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4
Q

What is the composition of the outer plaque of the hemidesmosome?

A

Integrin alpha6beta4, collagen XVII (cytoplasmic domain)

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5
Q

Define anchoring fibrils

A

Connections extending from lamina dense to collagen fibers in the dermis

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6
Q

What is the composition of anchoring fibrils?

A

Collagen VII

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7
Q

Define integrins

A

Cell adhesion molecules that connect the extracellular matrix to the cytoskeleton

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8
Q

What family is BPAG1-e a member of?

A

Plakins

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9
Q

What autoimmune conditions in dogs may result from autoantibodies against BPAG1?

A

Bullous pemphigoid, MMP

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10
Q

Which classes of adhesion molecules (CAMs) operate independent of Ca++?

A

integrins, IgCAMs

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11
Q

Which classes of adhesion molecules (CAMs) are dependent on Ca++?

A

cadherins, selectins

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12
Q

What class of adhesion molecules is present on leukocytes, allowing for leukocyte adhesion to the endothelium?

A

Integrins

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13
Q

What type of integrins bind leukocytes to the endothelium?

A

Beta2

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14
Q

What type of integrins bind T-cells to APCs?

A

Beta2

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15
Q

What beta2 chain is common to many integrins?

A

CD18

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16
Q

What is a primary role of beta3 integrins?

A

Clotting

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17
Q

What integrin on T-cells binds to ICAM-1 on APCs to allow for successful antigen recognition?

A

LFA-1

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18
Q

What integrin on endothelial cells is important for extravasation of leukocytes when bound?

A

ICAM-2 (bound by LFA-1 on neuts)

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19
Q

What does alpha6beta4 bind to extracellularly?

A

Laminin 332

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20
Q

In what layer(s) of the epidermis are integrins expressed?

A

Stratum basale only

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21
Q

What cell adhesion molecule is present on inflamed endothelial cells and binds VLA-4 to allow for generation of ROS?

A

VCAM-1 (CD106)

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22
Q

What are the classical type 1 cadherins?

A

E-cadherin, N-cadherin, P-cadherin

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23
Q

What cell types express N-cadherin?

A

Myocytes, neurons, fibroblasts

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24
Q

What are the classical type 2 cadherins?

A

VE-cadherin, M-cadherin

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25
Q

What cadherin becomes temporarily dysregulated to allow for diapedesis?

A

VE-cadherin

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26
Q

What is the major autoantigen in human PF?

A

Desmoglein-1

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27
Q

What is the major autoantigen in canine PF?

A

Desmocollin-1

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28
Q

What is the autoantigen in pemphigus vulgaris?

A

Desmoglein-3

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29
Q

Describe the locations of desmogleins 1-3 in the epidermis

A

Desmoglein-1: superficial epidermis
Desmoglein-2: basal layer
Desmoglein-3: deep epidermis

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30
Q

Name two major functions of selectins

A
  1. Mediate initial low-affinity adhesion of WBC to endothelium
  2. Lymphocyte homing
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31
Q

What cell types express P-selectin?

A

Endothelial cells, platelets

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32
Q

What structures within endothelial cells store P-selectin?

A

Weibel-palade bodies

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33
Q

What structures within platelets store P-selectin?

A

alpha granules

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34
Q

What activates P-selectin?

A

Histamine, thrombin

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35
Q

What cells express E-selectin?

A

Endothelial cells

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36
Q

What cytokines activate E-selectin?

A

IL-1, TNF, LPS

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37
Q

What cells express L-selectin?

A

Leukocytes (NOT endothelial cells)

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38
Q

What dog breed is reported to be associated with canine leukocyte adhesion deficiency (CLAD)?

A

Irish Setters

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39
Q

Describe the pathogenesis of canine leukocyte adhesion deficiency

A

Single missense mutation in beta2-integrin subunit gene –> cysteine replaced by serine at AA36 –>disulfide bond disruption in CD18 leading to altered structure and function–>recurrent infections despite normal or high WBC counts

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40
Q

How would you diagnose CLAD?

A

PCR

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41
Q

What cow breed has been reported in bovine leukocyte adhesion deficiency (BLAD)?

A

Holstein

42
Q

Describe the pathogenesis of BLAD

A

Point mutation in CD18 gene –> aspartic acid replaced by glycine –> dysfunctional integrin assembly and lack of WBC diapedesis

43
Q

What are the 4 major types of adhesive structures in the epidermis?

A

Adherens junctions, desmosomes, tight junctions, hemidesmosomes

44
Q

What histologic stain could be useful to highlight the BMZ?

A

PAS

45
Q

Name the layers of the basement membrane zone

A

Basal keratinocyte –> basal lamina (lamina lucida + lamina densa) –> sublamina densa

46
Q

Know the locations of various molecules in each layer of the BMZ

A
47
Q

Define the location of hemidesmosomes

A

Found along the ventral aspect of the basal keratinocyte

48
Q

Name the 4 components of a hemidesmosome

A

BPAG-1, plectin, integrin alpha6beta4, collagen XVII

49
Q

What is the molecular target of epidermolysis bullosa simplex in cattle and dogs?

A

KRT5

50
Q

What is the molecular target of epidermolysis bullosa simplex in cats?

A

KRT14

51
Q

What is the molecular target of epidermolysis bullosa simplex in Eurasier dogs?

A

Plectin

52
Q

What is the molecular target of junctional epidermolysis bullosa in cattle?

A

ITGA6 (integrin alpha6), ITGB4 (integrin beta4)

53
Q

What is the molecular target of junctional epidermolysis bullosa in sheep?

A

ITGB4 (integrin beta4)

54
Q

What would be considered the primary inflammatory cell infiltrate in bullous pemphigoid in dogs?

A

Eosinophils

55
Q

What is the target of bullous pemphigoid in dogs?

A

NC16A domain of collagen XVII

56
Q

Where does the split occur in junctional epidermolysis bullosa involving laminin 332?

A

above the lamina densa

57
Q

What does PAS stain in the lamina densa?

A

Nidogen and perlecan (proteoglycans)

58
Q

What component of the basement membrane zone forms a hexagonal meshwork?

A

Collagen IV (lamina densa)

59
Q

What is the main molecular target of epidermolysis bullosa acquisita in dogs?

A

Collagen VII (esp NC1 domain)

60
Q

What is unique in regards to EBA compared to other acquired AISBDs in dogs?

A

Age of onset- EBA has young onset (1-2 years though puppies may be affected), other AISBDs have 5-6 year of age onset

61
Q

The “floor” stains on indirect immunofluorescence on salt split mucosa. What is/are the most likely differential(s)?

A

EBA, JEBA

62
Q

The “roof”/epidermal side stains on indirect immunofluorescence on salt split mucosa. What is/are the most likely differential(s)?

A

BP

63
Q

There is “mixed” staining on indirect immunofluorescence on salt split mucosa. What is/are the most likely differential(s)?

A

MMP (usually roof stains but can be both)

64
Q

A ragged, intracellular split occurs on PAS staining of a suspect AISBD. What is the most likely differential?

A

EB simplex

65
Q

What is the major autoantigen in MMP?

A

Collagen XVII (BPAG2)
–BPAG1 and laminin 332 also reported

66
Q

What is the major autoantigen in JEBA?

A

Laminin 332

67
Q

What antigens stain on the floor of salt-split skin?

A

Laminin 332, collagen VI, collagen VII

68
Q

What antigens stain on the roof of salt-split skin?

A

K5, K14, plectin, BPAG1, BPAG2, integrin alpha6beta4

69
Q

What is the most common AISBD in dogs?

A

MMP

70
Q

What dog breed has a suspected genetic predisposition to EBA?

A

Great Dane

71
Q

What 3 collagen types are part of the BMZ?

A

Collagens XVII (hemidesmosome), IV (lamina densa), VII (sublamina densa)

72
Q

What do KIF attach to within the BMZ?

A

BPAG1 and plectin on inner plaque

73
Q

What does integrin alpha6beta4 bind to in the BMZ?

A

Inner plaque: BPAG1, plectin
Outer plaque: BPAG2
Lamina densa: laminin-332

74
Q

What collagen type makes up most of the lamina densa?

A

Collagen IV

75
Q

What collagen type makes up the anchoring fibrils that link the lamina densa to the sublamina densa?

A

Collagen VII

76
Q

What parts of the BMZ are secreted by dermal fibroblasts?

A

Collagen IV, Collagen VII, laminin 332, laminin 311, laminin 511

77
Q

Is epidermolysis bullosa cell-rich or cell-poor?

A

cell-poor

78
Q

What cells secrete MOST components of the BMZ?

A

basal keratinocytes

79
Q

What is another name for collagen XVII?

A

BPAG2

80
Q

What is considered a crucial step in hemidesmosome assembly?

A

Binding of integrin alpha6beta4 to laminin-332

81
Q

What structure of the BMZ looks like a cross?

A

Laminin-332

82
Q

Are footpads commonly affected in canine bullous pemphigoid?

A

No

83
Q

What breed is overrepresented in MMP?

A

GSD

84
Q

Are footpads typically involved in MMP?

A

No

85
Q

Is MMP rapidly or slowly progressive?

A

Slowly

86
Q

Is EBA rapidly or slowly progressive?

A

Rapidly

87
Q

Are footpads typically involved in EBA?

A

Yes- sloughing seen

88
Q

What is the primary inflammatory infiltrate in EBA?

A

Neutrophils

89
Q

In what layer of the BMZ would you find anchoring filaments?

A

Lamina lucida

90
Q

What is the major target in dystrophic epidermolysis bullosa?

A

Collagen VII

91
Q

What is the most common AISBD in humans?

A

Bullous pemphigoid

92
Q

Where is the salt split for EBA?

A

Dermal side (both sides rare)

93
Q

What is the pathogenesis of acquired junctional EB?

A

IgG autoantibodies to laminin 332

94
Q

Where is the salt split for acquired junctional EB?

A

Dermal side or both

95
Q

Where is the salt split for linear IgA bullous disease?

A

epidermal

96
Q

When does the onset of epidermolysis bullosa simplex occur?

A

immediately after birth

97
Q

Where do the blisters occur in junctional EB?

A

within the lamina lucida

98
Q

Where do blisters occur in dystrophic epidermolysis bullosa?

A

Beneath lamina densa

99
Q

What is the mutation in dystrophic epidermolysis bullosa?

A

COL7A1 (collagen VII)

100
Q

What is the inheritance pattern for dystrophic epidermolysis bullosa in cats, dogs, and ruminants?

A

Autosomal recessive (humans also have a dominant form)

101
Q

What is the PAS staining pattern for dystrophic EB?

A

BMZ attached to roof of cleft

102
Q
A

MK ACVD Exam (7 decks)

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