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FD9 > 15 - Epidermal and Dermal Adhesion > Flashcards

15 - Epidermal and Dermal Adhesion Flashcards

1
Q

Major cellular structures involved at cell-cell junctions in the epidermis

A

Desmosomes

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2
Q

Basement membrane adhesion complexes and related structures at the dermal-epidermal junction

A

Hemidesmosomes

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3
Q

Desmosome is also known as

A

Macula adherens

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4
Q

Major cell adhesion junction of the epidermis

A

Desmosomes

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5
Q

Anchor apposing keratinocyte cell surface membranes to the intercellular keratin intermediate filament network

A

Desmosome

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6
Q

Desmosomal cadherins

A

Desmogleins

Desmocollins

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7
Q

Plakins

A

Desmoplakin
Envoplakin
Periplakin

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8
Q

Armadillo family proteins

A

Plakoglobin

Plakophilins

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9
Q

Transmembrane proteins whose extracellular amino-terminal domains interact to form the trans-adhesive interface between cells, represented by the electron-dense midline of the desmoglea

A

Desmosomal cadherins

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10
Q

Contains the desmosomal cadherin cytoplasmic tails, with plakoglobin and the desmoplakin amino-terminal domain, and plakophilin

A

Outer dense plaque

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11
Q

Desmoplakin carboxyl-terminus interacts with keratin intermediate filaments, producing the

A

Inner dense plaque

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12
Q

Desmosomal cadherins mediate _____-dependent adhesion in a variety of epithelial tissues

A

Calcium

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13
Q

In humans, there are _____ desmoglein isoforms and _____ desmocollin isoforms

A

4

3

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14
Q

Expressed predominantly in the differentiated cells of the superficial epidermis

A

Dsg1
Dsg4
Dsc1

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15
Q

Expressed more strongly in the basal and/or suprabasal layers

A

Dsg2
Dsg3
Dsc2
Dsc3

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16
Q

Dsg: Largely limited to statified squamous epithelia in the skin

A

Dsg1

Dsg3

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17
Q

Dsg: Also strongly expressed in squamous cell carcinomas and other head and neck cancers, where it has been proposed as a potential molecular target for therapy

A

Dsg3

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18
Q

Dsg: Major desmoglein isofrom in most simple and transitional epithelia, as well as cardiac myocytes

A

Dsg2

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19
Q

Dsg: Prominent in desmosomes of the hair follicle, testis, and prostate

A

Dsg4

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20
Q

Dsc: Largely limited to skin and oral epithelia

A

Dsc1

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21
Q

Dsc: More widely expressed in most desmosome-containing epithelia and is the only desmocollin isoform in cardiac tissue

A

Dsc2

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22
Q

Dsc: Most strongly expressed in stratifies squamous epithelia of the skin and oropharynx

A

Dsc3

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23
Q

Desmosomal cadherins have been reported to engage in both homopholic and heterophilic interactions, although _____ interactions likely form the basis of epidermal intercellular adhesions

A

Heterophilic

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24
Q

Desmoglein 1 autoimmune target

A

PF
PV
IgA pemphigus (intraepidermal neutrophilic-type)

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25
Q

Desmoglein 1 genetic target

A

Striate PPK (AD)

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26
Q

Desmoglein 1 infectious target

A

SSSS

Bullous impetigo

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27
Q

Desmoglein 2 genetic target

A

ARVC (AD)

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28
Q

Desmoglein 3 autoimmune target

A

PV
PNP
IgA pemphigus (intraepidermal neutrophilic-type)

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29
Q

Desmoglein 4 autoimmune target

A

PF

PV

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30
Q

Desmoglein 4 genetic target

A

Hypotrichosis (AR)

Monilethrix (AR)

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31
Q

Desmocollin 1 autoimmune target

A

IgA pemphigus (subcorneal pustular dermatosis-type)

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32
Q

Desmocollin 2 genetic target

A

ARVC (AR and AD)

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33
Q

Desmocollin 3 autoimmune target

A

PV

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34
Q

Desmocollin 3 genetic target

A

Hypotrichosis (AR)

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35
Q

Desmoplakin I/II autoimmune target

A

PNP

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36
Q

Desmoplakin I/II genetic target

A

Striate PPK (AD)
Carvajal syndrome (AR)
Lethal acantholytic epidermolysis bullosa (AR)
Skin fragility-wooly hair syndrome (AR)

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37
Q

Plakoglobin genetic target

A

Naxos disease

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38
Q

Plakophilin 1 genetic target

A

Skin fragility and ectodermal dysplasia (AR)

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39
Q

Plakophilin 2 genetic target

A

ARVC (AD)

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40
Q

Corneodesmosin genetic target

A

Hypotrichosis simplex of the scalp (AD)

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41
Q

Diffuse PPK
Wooly hair
Left ventricular cardiomyopathy

A

Carvajal syndrome

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42
Q

PPK
Wooly hair
Nail dystrophy

A

Skin fragility-wooly hair syndrome

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43
Q

Diffuse PPK
Wooly hair
ARVC

A

Naxos disease

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44
Q

Desmoglein 1 is also targeted by hyperactive serine proteases in

A

Netherton syndrome

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45
Q

Plakoglobin is expressed

A

Throughout all layers of the epidermis and is ubiquitously expressed in all epithelia

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46
Q

Desmoglein _____ immunoreactivity is due to cross-reactivity with desmoglein
1 in pemphigus sera

A

4

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47
Q

No epidermal phenotype in affected patients

A

Dsg2

Dsc2

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48
Q

Directly bind to desmoplakin, and may also directly bind keratins and some desmosomal cadherins which is thought to aid in clustering and lateral stability of the desmosomal plaque

A

Plakophilins

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49
Q

Also known as gamma-catenin

A

Plakoglobin

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50
Q

Provides the major link between the keratin filaments and desmosomal plaque

A

Desmoplakin

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51
Q

Plakophilin _____ are both expressed throughout the epidermis, although plakophilin _____ is more predominant in basal compared to superficial epidermal keratinocytes

A

1 and 3

1

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52
Q

Plakophilin _____ is only expressed in the basal epidermal layer

A

2

53
Q

Currently, there are no known human diseases associated with plakophilin

A

3

54
Q

Envoplakin and periplakin are desmosomal plaque proteins expressed in the

A

Superficial layers of the epidermis

55
Q

Envoplakin and periplakin autoantibodies are characteristic of _____ sera

A

Paraneoplastic pemphigus

56
Q

Secreted glycoprotein that incorporates into corneodesmosomes and is also expressed in the inner root sheath of the hair follicle

A

Corneodesmosin

57
Q

Ultrastructurally, basement membranes most offen appear as trilaminar structures, consisting of a central electron-dense region, known as the _____, adjacent on either side to an apparently less-dense area known as the _____

A

Lamina densa

Lamina lucida

58
Q

The absence of distinguishable _____ in tumor biopsies is used as an indicator of malignancy

A

Basement membranes

59
Q

The DEJ can be divided into _____ distinct zones

A

3

60
Q

3 zones of the DEJ

A
  1. Keratin filament-hemidesmosome complex extending through the lamina lucida and lamina densa
  2. Lamina densa
  3. Sublamina densa
61
Q

May result from shrinkage of the cell surface away from the lamina densa due to dehydration

A

Lamina lucida

62
Q

The sublamina densa contains 2 readily distinguishable microfibrillar structures:

A
  1. Anchoring fibrils

2. Microfibrils

63
Q

Account for 40-65% of the total protein of the basement mebrane

A

Collage

64
Q

Ubiquitous components of basement membranes

A 
Collagen IV
Laminins
Nidogens
Heparan sulfate proteoglycans
Fibulins
65
Q

Collagen IV is a heterotrimer of 3 alpha chains. The _____ chains are ubiquitous.

A

Alpha 1 and 2

66
Q

The 6 chains of collagen IV are distributed in 3 major networks:

A
  1. Alpha1-alpha2
  2. Alpha3-alpha4-alpha5
  3. Alpha1-alpha2-alpha5-alpha6
67
Q

The chain compositions of collagen IV is determined by the

A

NC-1 domain

68
Q

Collagen IV networks in the glomerular BM

A

Alpha1-alpha2

Alpha3-alpha4-alpha5

69
Q

Collagen IV networks in the smooth muscle BM

A

Alpha1-alpha2

Alpha1-alpha2-alpha5-alpha6

70
Q

Collagen IV networks in the DEJ

A

Alpha1-alpha2 (dominates)

Alpha1-alpha2-alpha5-alpha6g

71
Q

Mutations in the genes encoding the alpha _____ chains cause pathologies in different organs, ranging from eye, brain, and muscle defect to small-vessel disease, which often underlies ischemic strokes and intracerebral hemorrhages and to the HANAC (hereditary angiopathy with nephropathy, aneurysms, and muscle cramps) syndrome

A

1 and 2

72
Q

Structural aberrations in the genes encoding the alpha _____ chains cause different forms of Alport syndrome

A

3, 4, 5 and 6

73
Q

Alport syndrome is a genetic disease characterized by

A

Nephritis

Deafness

74
Q

The _____ chain is the antigen recognized by the circulating autoantibodies in the Goodpasture syndrome

A

Alpha3(IV)

75
Q

Each laminin is a trimeric aggregate of _____ chain

A

1 alpha, beta and gamma

76
Q

Major laminin of the epidermal BM

A

Laminin-5

Now called laminin 332

77
Q

Mutations in the _____ laminin chains underlie junctional EB

A

Alpha3, beta3, gamma2

78
Q

Mutations in the _____ laminin chain cause congenital muscular dystrophy

A

Alpha2

79
Q

Relatively small molecules which bind laminins at a specific site within the gamma1 and the gamma3 chain, but also collagen IV, perlecan and fibulins

A

Nidogens 1 and 2

80
Q

These three proteoglycans are characteristically present in vascular and epithelial basement membranes:

A
  1. Perlecan
  2. Agrin
  3. Collagen XVIII
81
Q

Lack of _____ caused basement membrane deterioration in regions under increased mechanicsl stress, manifesting as lethal cardiac abnormalities and skin blistering

A

Perlecan

82
Q

Major heparan sulfata proteoglycan of neuromuscular junctions and renal tubular basement membranes

A

Agrin

83
Q

Considered to be a hybrid collagen-proteoglycan in various organs

A

Collagen XVIII

84
Q

Transmembrane heparan sulfate proteoglycans present on most cell types, including basal keratinocytes of the epidermis

A

Syndecans

85
Q

Family of highly conserved, calcium-binding extracellular matrix proteins
Believed to function as intermolecular bridges that stabilize supramolecular organization of extracellular matrix molecules, such as elastic fibers and basement membranes

A

Fibulins

86
Q

Genetic defects of the genes encoding fibulin 4 and 5 cause different forms of

A

Cutis laxa

87
Q

The 230-kDa bullous pemphigoid antigen 1, or BP230 is a protein with homology to _____, which bind intermediate filaments

A

Plakins

88
Q

Major component of the hemidesmosomal inner dense plaque

A

BP230

89
Q

Ablation of BP230 is associated with

A

Epidermolysis bullosa simplex

90
Q

Major antigen in bullous pemphigoid

A

180-kDa bullous pemphigoid antigen 2 or BP180

91
Q

180-kDa bullous pemphigoid antigen 2 or BP180 is a transmembrane collagen known as

A

Collagen XVII

92
Q

Mutations in collagen XVII cause

A

Junctional epidermolysis bullosa

93
Q

Plectin, another _____ homolog, is also a component of the hemidesmosome

A

Plakin

94
Q

Mutations of _____ result in epidermolysis bullosa simplex with progressive muscular dystrophy and, in some cases, epidermolysis bullosa with pyloric atresia

A

Plectin

95
Q

One key component of the hemidesmosome is the _____. It has a high affinity for laminin 332 and is essential to integration of the hemidesmosome with the underlying basement membrane

A

Integrin alpha6beta4

96
Q

Mutations in either alpha6 or beta4 chains result in

A

Junctional epidermolysis bullosa associated with pyloric atresia

97
Q

A member of the widely expressed cell surface transmembrane proteins of the tetraspanin family, _____, is also a component of the hemidesmosome. It forms complexes with alpha3beta1 and alpha6beta4 integrins at the basolateral surface of basal keratinocytes and stabilizes their functions

A

CD151

98
Q

In addition to the expression of _____ in several tissues like the kidney and the skin, its gene also encodes the MER2 blood group antigen on erythrocytes

A

CD151

99
Q

_____-negative patients presented with hereditary nephritis, sensorineural deafmess, pretibial epidermolysis bullosa, and beta-thalassemia minor

A

MER2

100
Q

In addition to the hemidesmosomal components, other adhesion molecules are known to be present at the basolateral aspect of basal keratinocytes, for example, _____, the receptor for the laminin 332-311 complex in the basement membrane between the hemidesmosomes, and another transmembrae collagen, type _____

A

Integrin alpha3beta1

XIII

101
Q

Mutations in the _____ gene cause a rare systemic disorder with interstitial lung disease, nephrotic syndrome, and epidemolysis bullosa

A

Integrin alpha3

102
Q

Disorder with skin blistering in infancy, progressive poikiloderma, sun sensitivity, and skin cancer

A

Kindler syndrome

103
Q

Mutation in Kindler syndrome

A

Kindlin-1

104
Q

The anchoring filaments contain _____ and the ectodomain of _____, 2 ligands that interact with each other

A

Laminin 332

Collagen XVII

105
Q

The major alpha3-containing laminin in the lamina densa between hemidesmosomes is probably the

A

Laminin 332-311 complex

106
Q

The N-termini of laminin 322 bind to _____, the main component of the anchoring fibrils in the sublamina densa that, in turn, binds tightly to ______ containing fibrils so that anchoring filaments and fibrils are connected to each other and to the dermis

A

Collagen VII

Collagen I

107
Q

Major component of the anchoring fibrils

A

Collagen VII

108
Q

The collagen _____ molecule is distinguished from other collagens in that it has a very long triple-helical domain, 450 nm in length

A

VII

109
Q

Anchoring fibrils are further stabilized by tissue _____, which catalyzes the formation of covalent crosslinks

A

Transglutaminase

110
Q

In the acquires form of epidermolysis bullosa, epidermolysis bullosa aquisita, and in bullous systemic lupus erythematosus, autoantibodies target mainly the

A

NC-1 domain of collagen VII

111
Q

Mutations in _____, the gene encoding _____, result in dystrophic epidermolysis bullosa

A

COL7A1

Collagen VII

112
Q

Collagen, a family of 28 different collagen types, are the most abundant proteins in the dermis and represent _____% of its dry weight and _____% of its volume

A

75

20-30

113
Q

All collagens have a basic structure of

A

3 alpha-chains, which have glycine as every third amino acid

114
Q

In the dermis, the classic, cross-branded fibrils recognizable in the electron microscope contain collagen

A

I, III, V, XII, and XIV

115
Q

In contrast, collagen _____ polymerizes into microfibrils

A

VI

116
Q

Other collagens are needed to provide cohesion of the epidermis or the vascular structures with the dermal extracellular matrix, for example, collagens _____ in the epidermal basement membrane or collagens _____ in the vascular basement membrane zone

A

VII, XIII, XVII, XXIII

VIII, XV, XVIII

117
Q

Provides dermal elasticity

A

Elastin

118
Q

Elastin molecules are rich in repetitive (hydrophilic/hydrophobic) sequences and are highly crosslinked

A

Hydrophobic

119
Q

Elastic fibers can be stretched by _____% or more, and still return to their original form

A

100

120
Q

Main componenrs of elastic fibers

A

Elastin (90%)

Microfibrils

121
Q

Negativelg charged polysaccharides that can bind large amounts of ions and water

A

Glycosaminoglycans

122
Q

Four different proteoglycan-bound glycosaminoglycans are known

A
  1. Chondroitin sulfate
  2. Dermatan sulfate
  3. Keratan sulfate
  4. Heparan sulfate
123
Q

Major proteoglycan in the dermis

Associated with elastic fibers and forms huge complexes with hyaluronic acid, which provides the skin with its tautness

A

Versican

124
Q

Ubiquitous glycosaminoglycan without protein core, a giant polysaccharide composed of N-acetylglucosamine/glucuronic acid disaccharides

A

Hyaluronic acid

125
Q

Majro heparan sulfate proteoglycan of basement membranes that can sequester and present growth factors to their receptors

A

Perlecan

126
Q

Hybrid collagen/proteoglycan found in epidermal and vascular basement membranes in the skin

A

Collagen XVIII

127
Q

Small leucine-rich proteoglycans
Multiple functions:
-Bind to collagen fibrils and regulate their assembly
-Bind TGF-beta
-Connected to immune reactions by signalling through toll-like receptors

A

Decorin

Biglycan

128
Q

Transmembrane and glypicans membrane-anchored heparan sulfate proteoglycans
Control cell adhesion to the matrix and interact with multiple extracellular proteins, including growth factors, and thereby regulate cell functions

A

Syndecans

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