Serpin H1 inhibitory domain (IPR033830)

Short name: Serpin_H1_domain

Overlapping homologous superfamilies

Domain relationships

  • Serpin domain (IPR023796)
    • Serpin H1 inhibitory domain (IPR033830)


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 [PMID: 26004778]. The serpin does not inhibit serine peptidases, unlike most serpins, but has been shown to inhibit the lysosomal cysteine endopeptidase cathepsin L [PMID: 12685865].

This entry represents the inhibitory domain of serpin H1.

GO terms

Biological Process

No terms assigned in this category.

Molecular Function

GO:0005518 collagen binding
GO:0004867 serine-type endopeptidase inhibitor activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.