Anti Collagen Alpha-1 (VII) Chain mAb (Clone BML39)

This gene encodes the alpha chain of type VII collagen. The type VII collagen fibril, composed of three identical alpha collagen chains, is restricted to the basement zone beneath stratified squamous epithelia. It functions as an anchoring fibril between the external epithelia and the underlying stroma. Mutations in this gene are associated with all forms of dystrophic epidermolysis bullosa. The inherited disease, dystrophic epidermolysis bullosa, is caused by recessive or dominant mutations in COL7A1. In the absence of mutations, however, an autoimmune response against type VII collagen can result in an acquired form of this disease called epidermolysis bullosa acquisita. Type VII collagen is also found in the retina; its function in this organ is unknown. Collagen, type VII, alpha 1 has been shown to interact with Laminin 5 and Fibronectin.

Source: Nagoya University Graduate School of Science Department of Life Science Cell Biology Group Lecturer Mr. Yoshiko Hirako

References:
1) Uematsu J., et al. Eur J Cell Biol., 84: 407-415 (2005).
2) Hirako Y., et al. J. Biol. Chem., 273:9711-9717 (1998).