Thrombospondin type-1 repeat superfamily

Definition

Thrombospondins are multimeric multidomain glycoproteins that function at cell surfaces and in the extracellular matrix milieu. They act as regulators of cell interactions in vertebrates. They are divided into two subfamilies, A and B, according to their overall molecular organisation. The subgroup A proteins TSP-1 and -2 contain an N-terminal domain, a VWFC domain, three TSP1 repeats, three EGF-like domains, TSP3 repeats and a C-terminal domain. They are assembled as trimer. The subgroup B thrombospondins, designated TSP-3, -4, and COMP (cartilage oligomeric matrix protein, also designated TSP-5) are distinct in that they contain unique N-terminal regions, lack the VWFC domain and TSP1 repeats, contain four copies of EGF-like domains, and are assembled as pentamers PMID:11687483. EGF, TSP3 repeats and the C-terminal domain are thus the hallmark of a thrombospondin. This repeat was first described in 1986 by Lawler and Hynes PMID:2430973. It was found in the thrombospondin protein where it is repeated 3 times. Now a number of proteins involved in the complement pathway (properdin, C6, C7, C8A, C8B, C9) PMID:2459396 as well as extracellular matrix protein like mindin, F-spondin PMID:10409509, SCO-spondin and even the circumsporozoite surface protein 2 and TRAP proteins of Plasmodium [[cite:PMID:10508153], [cite:PMID:1501644]] contain one or more instance of this repeat. It has been involved in cell-cell interaction, inhibition of angiogenesis PMID:10500044 and apoptosis PMID:9135017. The intron-exon organisation of the properdin gene confirms the hypothesis that the repeat might have evolved by a process involving exon shuffling PMID:1417780. A study of properdin structure provides some information about the structure of the thrombospondin type I repeat PMID:1868073. The TSP1 repeat structure has a disulfide-rich fold with all-β sheets, each with three antiparallel strands.

65 human proteins with this domain

Each is a reviewed human protein in the Seroatlas serome and a potential autoantibody target; this domain groups them into one antibody-relevant category. Every entry links to its own record.

Download CSV (65 proteins: gene, accession, name)

• ADAMTS1
• ADAMTS10
• ADAMTS12
• ADAMTS13
• ADAMTS14
• ADAMTS15
• ADAMTS16
• ADAMTS17
• ADAMTS18
• ADAMTS19
• ADAMTS2
• ADAMTS20
• ADAMTS3
• ADAMTS4
• ADAMTS5
• ADAMTS6
• ADAMTS7
• ADAMTS8
• ADAMTS9
• ADAMTSL1
• ADAMTSL2
• ADAMTSL3
• ADAMTSL4
• ADAMTSL5
• ADGRB1
• ADGRB2
• ADGRB3
• C6
• C7
• C8A
• C8B
• C9
• CCN1
• CCN2
• CCN3
• CCN4
• CCN5
• CCN6
• CFP
• CILP
• CILP2
• HMCN1
• ISM1
• ISM2
• PAPLN
• RSPO1
• RSPO2
• RSPO3
• SBSPON
• SEMA5A
• SEMA5B
• SPON1
• SPON2
• SSPOP
• THBS1
• THBS2
• THSD1
• THSD4
• THSD7A
• THSD7B
• THSD8
• UNC5A
• UNC5B
• UNC5C
• UNC5D