reproductive system
• mutants expressing the transgene are able to reproduce, in contrast to Col17a1-deficient mice
|
immune system
• following intraperitoneal injection with 1 mg/g total IgG from patients with bullous Pemphigoid (BM), treatment of mice with R1-GST results in marked reduction in BP autoantibodies
|
integument
N |
• mice show none of the outward abnormal manifestations that Col17a1-deficient mice show
|
• when injected with IgG directed against human COL17A1, mice display epidermal separation elicited by gentle skin friction at 48 hours post-injection
• there is dermal-epidermal separation in the lamina lucida between the plasma membrane of basal keratinocytes and the lamina densa
|
blistering
(
J:121695
)
• following intraperitoneal injection with 1 mg/g total IgG from patients with bullous Pemphigoid (BM), treatment of mice with R1-GST results in marked reduction of skin blisters
|
reddish skin
(
J:121695
)
• when injected with IgG directed against human COL17A1, mice develop diffuse erythema
|
skin lesions
(
J:121695
)
• mice have lesions showing inflammatory cell infiltrate including neutrophils and lymphocytes
• there is a linear deposition of human IgG along the dermal-epidermal juctions
• R1 is a peptide that suppresses the BM antibody complex; following intraperitoneal injection with 1 mg/g total IgG from patients with Bullous Pemphigoid (BM), treatment of mice with R1-GST results in marked reduction of skin fragility or blistering, and no subepidermal blistering is observed after treatment
• there is reduced human IgG deposition along dermal-epidermal junction after R1 treatment
|