Collagen XVII

Stains
Collagen XVII

Author: Kara Hamilton, M.S., Nat Pernick, M.D. (see Authors page)

Revised: 7 December 2015, last major update July 2013

Copyright: (c) 2003-2015, PathologyOutlines.com, Inc.

PubMed Search: collagen xvii

Table of Contents

Definition / general | Diagrams / tables | Immunofluorescence | Positive staining - normal | Positive staining - disease

Cite this page: Collagen XVII. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/stainscollagenXVII.html. Accessed July 12th, 2017.

Definition / general

Collagen XVII (BP180) is a transmembrane component of hemidesmosomes responsible for mediating epidermal adhesion to the dermal layer (OMIM #113811, Dermatol Clin 2010;28:61, Wikipedia)
Also called COL17, bullous pemphigoid antigen 2 (BP antigen II) and BP180
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)
Autoantibodies are associated with bullous pemphigoid, herpes gestationis and cicatricial pemphigoid (J Invest Dermatol 1996;106:141, Am J Pathol 2000;156:685, J Invest Dermatol 1997;109:573)
Regulates ameloblast differentiation; mutations are associated with enamel hypoplasia (Am J Pathol 2009;174:100)
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

Diagrams / tables

Basement membrane cell adhesion

Immunofluorescence

Fig A, part a: keratinocytes

Positive staining - 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 staining - disease

Invasive front of cutaneous squamous cell carcinoma (J Histochem Cytochem 2003;51:921)