 CCPDomain abundant in complement control proteins; SUSHI repeat; short complement-like repeat (SCR) | |
|---|---|
| SMART ACC: | SM000032 |
| Description: | The complement control protein (CCP) modules (also known as short consensus repeats SCRs or SUSHI repeats) contain approximately 60 amino acid residues and have been identified in several proteins of the complement system. A missense mutation in seventh CCP domain causes deficiency of the b subunit of factor XIII. |
| InterPro ACC: | IPR000436 |
| InterPro abstract: | The extracellular sushi domain is characterised by a consensus sequence spanning ~60 residues containing four invariant cysteine residues forming two disulfide-bridges (I-III and II-IV), a highly conserved tryptophan, and conserved glycine, proline, and hydrophobic residues [ PUBMED:2751824 ]. Sushi domains are known … expand |
| Family alignment: | View the Family alignment or the Alignment consensus sequence |
| There are 156 860 CCP domains in 32 570 proteins in SMART's NRDB database. | |
Taxonomic distribution of proteins containing CCP domains
The tree below includes only several representative species and genera. The complete taxonomic breakdown of all proteins containing CCP domains can be accessed here. Click the counts or percentage values to display the corresponding proteins.
Relevant references for this domain
Primary literature for the CCP domain is listed below. Automatically-derived, secondary literature is also available.
Disease genes where sequence variants are found in this domain
UniRef sequences and OMIM curated human diseases associated with missense mutations within the CCP domain.
| Protein | Description | Disease / phenotype |
|---|---|---|
| LYAM3_HUMAN | OMIM:173610 : Platelet alpha/delta storage pool deficiency | |
| APOH_HUMAN | OMIM:138700 : APOLIPOPROTEIN H; APOH | |
| F13B_HUMAN | OMIM:134580 : Factor XIIIB deficiency | |
| CO2_HUMAN | OMIM:217000 : C2 deficiency |
KEGG pathways involving proteins which contain this domain
This information is based on the mapping of SMART genomic protein database to KEGG orthologous groups. Percentages are related to the number of proteins containing a CCP domain which could be assigned to a KEGG orthologous group, and not all proteins containing CCP domains. Please note that proteins can be included in multiple pathways, ie. the numbers below will not add to 100%.