Anti Collagen Alpha-1(XVII) Chain (BP180/BPAG2) mAb (Clone C34)

Antibodies/Cytoskeleton/Primary Antibodies; Antibodies/Primary Antibodies/Cytoskeleton; Research Area/Cell Biology/Cell Adhesion & Migration/Primary Antibodies; Research Area/Cell Biology/Cytoskeleton/Primary Antibodies; Research Area/Cell Types/Epithelial Cells/Primary Antibodies; Research Area/Disease/Epidermolysis bullosa/Primary Antibodies; Research Area/Disease/Epithelial recurrent erosion dystrophy/Primary Antibodies; Research Area/Organelles & Subcellular Structures/Desmosomes/Primary Antibodies; Research Area/Cell Biology/Extracellular Matrix/Primary Antibodies

CAC

Hemidesmosomes are adhesive structures between cells and the extracellular matrix. They play a role in anchoring intermediate fibers to the extracellular basement membrane. Structurally, hemidesmosomes occur in two forms: Type I and Type II. Type I hemidesmosomes develop in stratified epithelia such as the epidermis. Its main components include the intracellular linker proteins Plectin and BPAG1, the adhesion receptor integrin α 6β 4 and collagen type BP180/XVII. Type II hemidesmosomes occur in blood vessels, Schwann cells, and digestive tract epithelia as a simplified form of Type I hemidesmosomes, consisting only plectin and integrin α 6β 4. The hemidesmosomal adhesion receptor is normally associated with Laminin 5 in the basement membrane. Furthermore, Laminin 5 (of which Laminin gamma 2 is a subunit) is linked to collagen fibers in the dermis via type VII collagen. Genetic deletion of hemidesmosome-related proteins causes various forms of epidermolysis bullosa, highlighting their importance in promoting adhesion between the epidermis and the basement membrane.
Unlike most collagens, collagen XVII is a transmembrane protein. Collagen XVII is a structural component of hemidesmosomes, multiprotein complexes at the dermal-epidermal basement membrane zone that mediate adhesion of keratinocytes to the underlying membrane. Mutations in this gene are associated with both generalized atrophic benign and junctional epidermolysis bullosa.
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