Reactome: A Curated Pathway Database

Anchoring fibril formation (R-HSA-2214320) [Homo sapiens]


Collagen VII forms anchoring fibrils, composed of antiparallel dimers that connect the dermis to the epidermis (Bruckner-Tuderman 2009, Has & Kern 2010). During fibrillogenesis, the nascent type VII procollagen molecules dimerize in an antiparallel manner. The C-propeptide is then removed by Bone morphogenetic protein 1 (Rattenholl et al. 2002) and the processed antiparallel dimers laterally aggregate (Villone et al. 2008, Gordon & Hahn 2010).

Literature References
pubMedId Title Journal Year
19945621 Type VII collagen: the anchoring fibril protein at fault in dystrophic epidermolysis bullosa Dermatol Clin 2010