Domain

Thrombospondin-5, coiled coil region (IPR039081)

Short name: TSP-5_cc

Overlapping homologous superfamilies

None.

Domain relationships

Description

This entry represents the N-terminal coiled coil region of TSP-5, also known as cartilage oligomeric matrix protein (COMP). It forms a pentameric left-handed coiled coil (COMPcc) with a channel that is a unique carrier for lipophilic compounds [PMID: 23133613]. It is known to bind hydrophilic signaling molecules such as vitamin D3 and vitamin A, making it a possible targeted drug delivery system [PMID: 12426368]. TSP-5/COMP is expressed in all types of cartilage as well as in the vitreous of the eye, tendons, vascular smooth muscle cells, and heart. The pentamer is stabilized by inter-subunit disulfide bonds formed between cysteine residues adjacent to the C-terminal end of the coiled coil region [PMID: 8820492].

TSP-5 is essential for modulating the phenotypic transition of vascular smooth muscle cells and vascular remodeling. Mutations in TSP-5 result in two different inherited chondrodysplasias and osteoarthritic phenotypes: pseudoachondroplasia and multiple epithelial dysplasia [PMID: 15094116]. Deficiency of TSP-5 causes dilated cardiomyopathy (DCM), a common cause of congestive heart failure [PMID: 18193163]. Early increase in serum TSP-5 is associated with joint damage progression in patients with rheumatoid arthritis, thus representing a novel indicator of an activated destructive process in the joint [PMID: 21875984].

Contributing signatures

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