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Vet Derm Review > Croatia - Basement Membrane > Flashcards

Croatia - Basement Membrane Flashcards

1
Q

Autoimmune disease resulting from autoantibodies against BP230 or BP180?

A

bullous pemphigoid

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2
Q

T/F: Integrins are transmembrane proteins.

A

True - connect the extracellular matrix to the cytoskeleton of the cell

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3
Q

What are the anchoring filaments?

A

connect hemidesmosomes to anchoring fibrils throughout the lamina lucida

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4
Q

Where does collagen XVII attach?

A

hemidesmosome outer plaque [integrin beta-4, BPAG1-3]
non-collagenous terminus binds to integrin alpha-6
-laminin-5 (Lm3A32) in lamina densa

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5
Q

What is the most important laminin isoform present in the epidermal basement membrane

A

Laminin-5 (alpha-3/beta-3/gamma-2)

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6
Q

What plakins are found in the cornified envelope?

A

envoplakin, periplakin

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7
Q

What are intermediate filaments composed of?

A

keratin-5 and keratin-14

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8
Q

To what does Laminin-5 bind?

A

Integrin alpha-6/beta-4
Collagen VII
Collagen XVII

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9
Q

Keratin-14 is what type of keratin (type 1 or 2)?

A

Type 1 = small, acidic

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10
Q

Autoimmune disease resulting from autoantibodies against Collagen VII?

A

Epidermolysis Bullosa Acquisita

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11
Q

T/F: The lamina lucida is a fixation artefact and does not exist in vivo.

A

True

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12
Q

Collagen IV makes up what structure of the hemidesmosome?

A

lamina densa

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13
Q

Mutations in genes that encode for keratin filaments OR plectins can result in what clinical disease?

A

epidermolysis bullosa simplex

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14
Q

Autoimmune disease resulting from autoantibodies against BP230, BP180 (NC16A), Laminin-5?

A

Mucous membrane pemphigoid

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15
Q

Mutations in genes for Collagen IV cause what clinical disease(s)?

A

none recognized in animals
Autoimmune Subepidermal Blistering Disease with Renal Insufficiency (humans) – collagen IV located in glomerulus of kidneys

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16
Q

Genetic disease(s) resulting from dysfunction of Collagen VII?

A
  • Dominant Dystrophic Epidermolysis Bullosa (humans)
  • Recessive Dystrophic Epidermolysis Bullosa (humans, white alpine/Assaf sheep, Rotes Hohenvieh/Voderwald cattle, dogs, cats)
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17
Q

Mutations in genes that encode for Integrins or Laminins can result in what clinical disease?

A

Junctional Epidermolysis Bullosa

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18
Q

What are anchoring FIBRILS?

A

extend from lamina densa to dermal banded collagen fibers and to rare anchoring plaques, can loop back to the lamina densa

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19
Q

Mutations in the gene for integrin alpha-6/beta-4 can result in what clinical disease (reported in Charolais cattle)?

A

junctional epidermolysis bullosa

-lack the molecule to anchor the keratin filaments to the basement membrane (integrins)

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20
Q

Autoimmune disease resulting from autoantibodies against Laminin-5?

A

Acquired Junctional Epidermolysis Bullosa

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21
Q

What is the function of plakins?

A

they are cytolinkers – link cytoskeletal networks (intermediate filaments, actin, tubulin) to each other and to membrane-associated adhesion structures such as desmosomes and hemidesmosomes

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22
Q

What are the two keratins usually present in the stratum basale keratinocytes?

A

keratin-5 and keratin-14

23
Q

What plakins are found in the dermis?

A

desmoplakin I & II

24
Q

What is the purpose of intermediate filaments?

A

maintain the integrity and structure of basal epidermal cells

25
Q

Keratins always form heterodimers with what two types of keratin?

A

type 1 = small, acidic

type 2 = large, basic

26
Q

Autoimmune condition(s) that targets Collagen VII?

A

*Epidermolysis Bullosa Acquisita

Type I Bullous Systemic Lupus Erythematosus (develop EBA as part of SLE) – reported in humans & 1 dog

27
Q

What are focal contacts?

A

inter-hemidesmosomal adhesion structures that mostly functional during wound healing in which epidermal migration occurs

28
Q

Mutations in Laminin-5 result in what clinical disease(s)?

A

Junctional Epidermolysis Bullosa – Generalized or Localized–humans, dogs, horses, cattle

29
Q

_______ are a family of adhesion molecules that usually link the actin cytoskeleton to molecules of the extracellular matrix.

30
Q

What is the outer plaque of the hemidesmosome composed of?

A

integrin alpha-6/beta-4 + cytoplasmic domain (aminal terminus) of collagen XVII (BPAG2, BP180)

31
Q

Collagen XVII is also known as what?

A

BPAG2, BP180

bullous pemphigoid antigen 2– but actually most important in disease

32
Q

Mutations in the gene that encodes for BPAG1-e/BP230 (DST/BPAG1) can cause what clinical disease(s)?

A 
Bullous Pemphigoid (rare variant)
Mucous Membrane Pemphigoid (rare variant)
33
Q

What are the major components of focal contacts?

A 
on the keratinocyte membrane:
-integrin alpha-2/beta-1
-integrin alpha-3/beta-1
-integrin alpha-5/beta-1
on the lamina densa:
-laminin isoforms (3A32, 3A11)
-collagen XXIII
-nidogens
-fibulins
34
Q

What is the inner plaque of the hemidesmosome composed of?

A

plectin + BPAG1-e

35
Q

Why is the basement membrane highlighted with PAS stain? (i.e. what molecule is being stained)

A

sugars in perlecan are being stained

Perlecans link Collagen IV & laminin-alpha chains

36
Q

What are intermediate filaments?

A

connect the nuclear membrane to hemidesmosomes on keratinocyte membrane

37
Q

What are the main epidermal basement membrane collagens?

A

collagen XVII, IV, and VII

38
Q

What are the plakins found in the hemidesmosomes?

A

BPAG1e, epiplakin, plectin I & II

39
Q

Keratin-5 is what type of keratin (type 1 or 2)?

A

Type 2 = large, basic

40
Q

What are the anchoring filaments composed of?

A

intact and shed extracellular domain (carboxyl terminus) of collagen XVII (BPAG2, BP180)

41
Q

Autoimmune disease(s) directed against Laminin-5?

A 
Mucous Membrane (cicatricial) Pemphigoid
Acquired Junctional Epidermolysis Bullosa
42
Q

Mutations in KRT5 (gene encoding for keratin-5) can lead to what disease(s)?

A

Basal epidermolysis bullosa (EB) simplex variants

43
Q

Autoantibodies against collagen XVII can result in what clinical diseases?

A 
bullous pemphigoid (against NC16A segment)
mucous membrane (cicatricial) pemphigoid (against NC16A segment, col15 segment, or carboxyl terminus of collagen XVII)
Linear IgA bullous disease
44
Q

Mutations in the gene that encodes for plectin (PLEC/PLEC1) can cause what clinical disease(s)?

A
  • Epidermolysis Bullosa Simplex and Laminitis (horses)

- Epidermolysis Bullosa Simplex (EBS Plectin) - Eurasier dogs

45
Q

What are anchoring FIBRILS composed of?

A

collagen VII

46
Q

What is the lamina densa composed of?

A
  • collagen IV
  • laminin isoforms (laminin-5 [Lm332], -6 [LM311], -10 [Lm511])
  • perlecan (heparan-sulfate proteoglycan)
  • nidogens
  • fibulins
  • other components
47
Q

Integrin alpha-6/beta-4 connects ______ ______ via hemidesmosomes to ______.

A

intermediate filaments; laminin-5

48
Q

What are hemidesmosomes?

A

half-desmosomes situated on the internal aspect of the basal side of stratum basale keratinocytes
-divided into outer & inner plaques

49
Q

Mutations in genes that encode for Collagen VII can result in what clinical disease?

A

Dystrophic Epidermolysis Bullosa

50
Q

What is a collagen domain?

A

glycine-aminoacid1-aminoacid2 triad

51
Q

What collagen type is the sole member of the anchoring fibril-forming subfamily?

A

Collagen VII – anchors hemidesmosomes to dermal collagens

52
Q

How are keratins formed into intermediate filaments?

A

two keratins form heterodimers, then two dimers join into tetramers –> tetramers assemble into profilmanets –> protofibrils –> intermediate filaments

53
Q

Humans and a single case report of a dog with paraneoplastic pemphigus are reported to have auto-antibodies against what molecule?

A

epiplakin (plakins)

Vet Derm Review (44 decks)

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