Epidermal Proteins Flashcards

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1
Q

K1/K10

A

Suprabasal keratinocytes.

EHK (bullous CIE)
Unna Thost PPK (non-epidermolytic PPK)

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2
Q

K1/K9

A

Palmoplantar suprabasal keratinocytes

Vorner PPK (epidermolytic PPK)

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3
Q

K2/K10

A

Granular and upper spinous layer

Icthyosa Bullosa of Siemens

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4
Q

K3/K12

A

Cornea

Meesman corneal dystrophy

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5
Q

K4/K13

A

Mucosal epithelium

White sponge nevus

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6
Q

K5/K14

A

Basal keratinocytes

Dowling-Degos disease
EBS

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7
Q

K6a/K16

A

Outer root sheath

Pachyonychia Congenita Type 1
Hyperproliferative conditions (psoriasis)
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8
Q

K6b/K17

A

Nail bed

Pachyonychia Congenita Type 2

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9
Q

K8/K18

A

Simple epithelium

Cryptogenic cirrhosis

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10
Q

K81/K86

A

Hair

Monilethrix

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11
Q

K13

A

Warts in transplant recipients

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12
Q

K19

A

Stem cells

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13
Q

Plakoglobin

A

Desmosome

Naxos syndrome

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14
Q

Desmocolin

A

Desmosome

Subcorneal pustular dermatosis

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15
Q

Desmoplakin

A

Desmosome

Carvajal syndrome

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16
Q

Desmoglein 1

A

Desmosome

Pemphigus Folliaceous
Striate PPK

17
Q

Desmoglein 3

A

Desmosome

Pemphigus Vulgaris

18
Q

Plakophilin

A

Desmosome

Ectodermal dysplasia with skin fragility

19
Q

B-catenin

A

Adherens junctions

Pilomatricomas
Myotonic dystrophy

20
Q

GJB2

A

Connexin 26
Gap Junction

KID Syndrome
Vohwinkel’s PPK

21
Q

GJB6

A

Connexin 30
Gap Junction

Hidrotic ectodermal dysplasia (Clouston)

22
Q

GJB3

GJB4

A

Connexin 30.3
Connexin 31
Gap Junction

Erythrokeratododerma Variabilis (Mendes de Costa)

23
Q

a6B4 integrin

A

Hemidesmosome

Junctional EB with pyloric atresia
Cicatricial pemphigoid (ocular)
24
Q

Plectin

A

Hemidesmosome

EB Simplex with muscular dystrophy

25
Q

BPAG1

BPAG2

A

Hemidesmosome

Bullous pemphigoid

26
Q

Futcher’s lines

A

lines of pigmentary demarcation (ventral and dorsal surfaces, like front and back of hand)

27
Q

Lipid and calcium changes moving up the epidermis

A
increasing calcium (more desmosomes)
increasing sphingolipids (important for differentiation)
decreasing phospholipids
28
Q

Time for epidermal turnover

Time for stratum corneum turnover

A

45-60 days

2 weeks

29
Q

Type I and Type II keratins

A

Type 1 = acidic
epithelial K9-K28
hair K31-K40

Type 2 = basic
epithelial K1-K8, K71-K80
hair K81-K86

30
Q

Lamellar Granules

A

Stratum spinosum

Phospholipids, cholesterol sulfate, glucosylceramides
Proteases
Human beta defensin 2

Decreased, harlequin icthyosis
No steroid sulfatase, x-linked icthyosis
Increased (but abnormal), CIE

31
Q

Desmosome Junctions

A

Ca-depending, scaffolding, stress bearing

Desmosomal Cadherins (transmembrane)
Desmoglein 1/3
Desmocolin 1/2

Plaque proteins
Plakoglobin (gamma catenin) - also in adherens junctions
Desmoplakin 1/2
Envoplakin

32
Q

Adherens Junctions

A

Adhesion
Transmembrane, classical cadherins (E and P)

alpha, beta, and gamma cadherin