Authors: Kara Hamilton, M.S., Nat Pernick, M.D., PathologyOutlines.com, Inc.
Revised: 22 September 2012, last major update March 2010
Copyright: (c) 2010-2012, PathologyOutlines.com, Inc.
● Collagen XVII (BP180) is a transmembrane component of hemidesmosomes responsible for mediating epidermal adhesion to the dermal layer (OMIM 113811)
● Also called COL17, bullous pemphigoid antigen 2 (BP antigen II) and BP180
Pathophysiology / Clinical
● Mediates epidermal adhesion to the dermal layer (Dermatol Clin 2010;28:61); mutations / deficiency are associated with non-Herlitz junctional epidermolysis bullosa (J Invest Dermatol 2005;125:1112, OMIM 226650) and abnormal tooth formation of amelogenesis imperfecta (Int J Mol Med 2006;18:333)
● Regulates ameloblast differentiation; mutations are associated with enamel hypoplasia (Am J Pathol 2009;174:91)
● Lost during normal keratinocyte maturation but reexpressed upon malignant transformation (Appl Immunohistochem Mol Morphol 2008;16:433)
● May play a role in trophoblast invasion, and therefore a role in placental formation
Drawings, University of Oulu, Finland
Positive stains (normal)
● Stratified and complex epithelia such as skin, mucous membranes, eye
● First trimester and term syncytial and cytotrophoblastic cells of normal placenta and hemidesmosomes of amniotic epithelium (Matrix Biol 2008;27:190)
Positive stains (disease)
● Invasive front of cutaneous squamous cell carcinoma (J Histochem Cytochem 2003;51:921)
Fig A, part a: Keratinocytes
Fig 4a: lack of Collagen XII staining
Fig 4b: partial lack of staining adjacent to normal skin with intense staining
End of Stains > Collagen XVII
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