Colligin 1 (also known as SERPINH1; MEROPS identifier I04.035) is a chaperone exclusive for collagen folding in the ER. Mutations in the gene cause a severe form of osteogenesis imperfecta, a connective tissue disease characterised by bone fragility and increased risk of fractures in dogs []. The serpin does not inhibit serine peptidases, unlike most serpins, but has been shown to inhibit the lysosomal cysteine endopeptidase cathepsin L [].This entry represents the full sequence of serprin H1.
