Anti-Collagen XVII/COL17A1 Antibody Picoband® Fluoro488 Conjugated

Collagen XVII, previously called BP180, is a transmembrane protein which plays a critical role in maintaining the linkage between the intracellular and the extracellular structural elements involved in epidermal adhesion. It is mapped to 10q25.1. This gene encodes the alpha chain of type XVII collagen. 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. Two homotrimeric forms of type XVII collagen exist. The full length form is the transmembrane protein. A soluble form, referred to as either ectodomain or LAD-1, is generated by proteolytic processing of the full length form.

Collagen alpha-1 (XVII) chain; 180 kDa bullous pemphigoid antigen 2; Bullous pemphigoid antigen 2; 120 kDa linear IgA dermatosis antigen; Linear IgA disease antigen 1; LAD-1; 97 kDa linear IgA bullous dermatosis antigen; 97 kDa LAD antigen; 97-LAD; Linear IgA bullous disease antigen of 97 kDa; LABD97; COL17A1; BP180; BPAG2

A synthetic peptide corresponding to a sequence at the C-terminus of human Collagen XVII/COL17A1, which shares 83.3% and 91.3% amino acid (aa) sequence identity with mouse and rat Collagen XVII/COL17A1, respectively.

Detected in skin. In the cornea, it is detected in the epithelial basement membrane, the epithelial cells, and at a lower level in stromal cells. Stratified squamous epithelia. Found in hemidesmosomes. Expressed in cornea, oral mucosa, esophagus, intestine, kidney collecting ducts, ureter, bladder, urethra and thymus but is absent in lung, blood vessels, skeletal muscle and nerves.

May play a role in the integrity of hemidesmosome and the attachment of basal keratinocytes to the underlying basement membrane. The 120 kDa linear IgA disease antigen is an anchoring filament component involved in dermal-epidermal cohesion. Is the target of linear IgA bullous dermatosis autoantibodies.

1. Chavanas, S., Gache, Y., Tadini, G., Pulkkinen, L., Uitto, J., Ortonne, J. P., Meneguzzi, G. A homozygous in-frame deletion in the collagenous domain of bullous pemphigoid antigen BP180 (type XVII collagen) causes generalized atrophic benign epidermolysis bullosa. J. Invest. Derm. 109: 74-78, 1997. Note: Erratum: J. Invest. Derm. 109: 613 only, 1997. 2. Copeland, N. G., Gilbert, D. J., Li, K., Sawamura, D., Giudice, G. J., Chu, M.-L., Jenkins, N. A., Uitto, J. Chromosomal localization of mouse bullous pemphigoid antigens, BPAG1 and BPAG2: identification of a new region of homology between mouse and human chromosomes. Genomics 15: 180-181, 1993. 3. Darling, T. N., Koh, B. B., Bale, S. J., Compton, J. G., Bauer, J. W., Hintner, H., Yancey, K. B. A deletion mutation in COL17A1 in five Austrian families with generalized atrophic benign epidermolysis bullosa represents propagation of an ancestral allele. J. Invest. Derm. 110: 170-173, 1998.