Biology of BMZ

Question 1

4 layers of BMZ

Answer 1

basal keratinocytes
lamina lucida
lamina dena
sublamina densa

Question 2

structure a/w each layer of BMZ

Answer 2

basal keratinocytes – hemidesmosome
lamina lucida– anchoring filaments
lamina densa – anchoring plaque
sublamina densa – anchoring fibril

Question 3

Macromolecues a/w hemidesomsome

Answer 3

BPAG1 (230 kDa)
BPAG2 (180 kDa)
a6B4 integrin
plectin

Question 4

macromolecules a/w lamina lucida

Answer 4

laminin

portion of BPAG2

Question 5

macromolecules a/w lamina densa

Answer 5

type IV collagen
laminins
heparin sulfate

Question 6

macromolecules a/w sublamina densa

Answer 6

type VII collagen
fibrillin
type IV collagen and I and III

Question 7

NC16A domain of BPAG2 is target for what dz’s

Answer 7

BP
pemphigus gestationis
linear IgA bullous dermatosis

Question 8

carboxy terminus of BPAG2 is target for what dz

Answer 8

CP

Question 9

dz a/w autoantibody to beta4 (part of integrin) vs mutation in beta4

Answer 9

Ab = ocular CP
mut = JEB with pyloric atresia

Question 10

what is the weakest part of BMZ

Answer 10

lamina lucida

Question 11

type VII collagen Abs in what dz’s vs mut

Answer 11

Abs: EBA, bullous SLE
Mut: dystrophic EB