Collagen type VIII degradation by ELANE

Stable Identifier
R-HSA-2482180
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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Collagen type VIII is a short chain, network-forming collagen,thought to play a role in tissue remodeling and repair (Shuttleworth 1997, Weitkamp et al. 1999). There are two alpha chain subtypes, found in a ratio of two alpha-1 to one alpha-2 chains (Mann et al. 1990) in the typical collagen heterotrimer. Studies suggest that type VIII collagen is a major component of the hexagonal lattice seen in Descemet’s membrane (Mann et al. 1990). Mutations in both alpha chains have been associated with Fuchs endothelial corneal dystrophy (FECD), a degenerative disease of the corneal endothelium (Jun et al. 2012). Collagen type VIII can be degraded by neutrophil elastase (ELANE, ELA2; Kittelberger et al. 1992).

Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
serine-type endopeptidase activity of ELANE [extracellular region]
Physical Entity
Activity
Inferred From
Authored
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Created