Role of the Bullous Pemphigoid Antigen 180 (BP180) in the Assembly of Hemidesmosomes and Cell Adhesion—Reexpression of BP180 in Generalized Atrophic Benign Epidermolysis Bullosa Keratinocytes

1998 ◽  
Vol 239 (2) ◽  
pp. 463-476 ◽  
Author(s):  
Luca Borradori ◽  
Stéphane Chavanas ◽  
Roel Q.J. Schaapveld ◽  
Laurent Gagnoux-Palacios ◽  
Jero Calafat ◽  
...  
Keyword(s):  
Cell Adhesion ◽  
Bullous Pemphigoid ◽  
Epidermolysis Bullosa ◽  
Bullous Pemphigoid Antigen

scholarly journals Gambaran Dermal-Epidermal Junction Pada Bullous Pemphigoid Dan Epidermolysis Bullosa Acquisita

Medicinus ◽  
2018 ◽  
Vol 6 (3) ◽  
Author(s):  
Caroline Budiman ◽  
Mutia Devi ◽  
HM Athuf Thaha
Keyword(s):  
Autoimmune Disease ◽  
Basement Membrane ◽  
Bullous Pemphigoid ◽  
Epidermolysis Bullosa ◽  
Intermediate Filament ◽  
Autoimmune Disorder ◽  
Epidermolysis Bullosa Acquisita ◽  
Bullous Pemphigoid Antigen ◽  
Xvii Collagen

Epidermis and dermis of the skin attach each other because of the role of a structure which is called dermal-epidermal junction. The dermal-epidermal junction can be divided into three distinct zones named hemidesmosome-keratin intermediate filament complex, basement membrane, and anchoring fibril. Each part of the distinct zone has significant components for the integrity of the skin. Bullous pemphigoid is an autoimmune disorder caused by autoantibody against a 180-kDa molecule now called Bullous Pemphigoid Antigen 2 (BPAG2) or type XVII collagen within basement membrane. To date, the patophysiology and the components of dermal-epidermal junction that induce the blister formation remain unclear. Epidermolysis bullosa acquisita is an autoimmune disease with sub-epidermal blistering associated with autoimmunity to the collagen within anchoring fibrils. The pathophysiology and etiology—again—remain unknown.

scholarly journals Beta4 integrin is required for hemidesmosome formation, cell adhesion and cell survival.

1996 ◽  
Vol 134 (2) ◽  
pp. 559-572 ◽  
Author(s):  
J Dowling ◽  
Q C Yu ◽  
E Fuchs
Keyword(s):  
Cell Adhesion ◽  
Cell Survival ◽  
Schwann Cells ◽  
Basal Lamina ◽  
Epidermolysis Bullosa ◽  
Junctional Epidermolysis Bullosa ◽  
Human Disorder ◽  
Stratified Epithelia

The integrin heterodimer alpha 6 beta 4 is expressed in many epithelia and in Schwann cells. In stratified epithelia, alpha 6 beta 4 couple with BPAG1-e and BPAG2 to form hemidesmosomes, attaching externally to laminin and internally to the keratin cytoskeleton. To explore the function of this atypical integrin, and its relation to conventional actin-associated integrins, we targeted the removal of the beta 4 gene in mice. Tissues that express alpha 6 beta 4 are grossly affected. Stratified tissues are devoid of hemidesmosomes, display only a very fragile attachment to the basal lamina, and exhibit signs of degeneration and tissue disorganization. Simple epithelia which express alpha 6 beta 4 are also defective in adherence, even though they do not form hemidesmosomes. In the absence of beta 4, alpha 6 is dramatically downregulated, and other integrins do not appear to compensate for the loss of this heterodimer. These data have important implications for understanding integrin function in cell-substratum adhesion, cell survival and differentiation, and for understanding the role of alpha 6 beta 4 in junctional epidermolysis bullosa, an often lethal human disorder with pathology similar to our mice.

Mosaic expression of uncein and 180-kDa bullous pemphigoid antigen in generalized atrophic benign epidermolysis bullosa

1997 ◽  
Vol 136 (5) ◽  
pp. 757-761 ◽  
Author(s):  
Y. MATSUMURA ◽  
Y. HORIGUCHI ◽  
K. TODA ◽  
H. FUJII ◽  
S. KORE-EDA ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Epidermolysis Bullosa ◽  
Bullous Pemphigoid Antigen ◽  
Mosaic Expression

Mutations in the 180–kD bullous pemphigoid antigen (BPAG2), a hemidesmosomal transmembrane collagen (COL17A1), in generalized atrophic benign epidermolysis bullosa

1995 ◽  
Vol 11 (1) ◽  
pp. 83-86 ◽  
Author(s):  
John A. McGrath ◽  
Biljana Gatalica ◽  
Angela M. Christiano ◽  
Kehua si ◽  
Katsushi Owaribe ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Epidermolysis Bullosa ◽  
Bullous Pemphigoid Antigen
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