Search tips
Search criteria 


Logo of molpathLink to Publisher's site
Mol Pathol. 2003 April; 56(2): 127–128.
PMCID: PMC1187305

Proposal for a unified CCN nomenclature


A proposal is put forth to unify the nomenclature of the CCN family of secreted, cysteine rich regulatory proteins. In the order of their description in the literature, CCN1 (CYR61), CCN2 (CTGF), CCN3 (NOV), CCN4 (WISP-1), CCN5 (WISP-2), and CCN6 (WISP-3) constitute a family of matricellular proteins that regulate cell adhesion, migration, proliferation, survival, and differentiation, at least in part through integrin mediated mechanisms. This proposal is endorsed by the International CCN Society and will serve to eliminate confusion from the multiple names that have been given to these molecules.


The members of a family of cysteine rich matricellular proteins have recently emerged as multifunctional regulators that control diverse cellular processes and play important roles in vascular and skeletal development.1 Known as CCN proteins, members of this protein family are characterised by an N-terminal secretory signal, followed by four structural domains with partial sequence identity to insulin-like growth factor binding proteins, von Willebrand factor type C repeat, thrombospondin type 1 repeat, and a C-terminus with sequence similarity to the C-termini of von Willebrand factor, mucin, and slit.2–5 The acronym CCN comes from the first three members of the family reported, namely CYR61 (cysteine rich 61), CTGF (connective tissue growth factor), and NOV (nephroblastoma overexpressed).6–8 Other members of the CCN family have also been identified, bringing the total number of known CCN proteins to six.9

Genes encoding CCN proteins have also been identified in other studies, either based on their differential expression in various tissues, tumour cell lines, or upon induction by growth factors or morphogens. Consequently, a variety of other names have been assigned to these genes that reflected the context of these studies. During the international workshop on the CCN family of genes in Saint-Malo, France, it was recognised that a unified nomenclature will serve to eliminate confusion in the literature.1 A consensus was reached to propose a unifying nomenclature for the CCN family, numbering them CCN1 through to CCN6 in the order in which they were described in the literature. Thus, CYR61 will be designated CCN1, CTGF as CCN2, NOV as CCN3, and WISP-1–3 as CCN4–6 (table 1 [triangle]). This proposed nomenclature is endorsed by the International CCN Society ( The board of the International CCN Society will be acting as a nomenclature committee for the attribution of CCN names to new member genes of the CCN family yet to be discovered.

Table 1
Nomenclature proposal


1. Perbal B, Brigstock DR, Lau LF. Report on the second international workshop on the CCN family of genes. Mol Pathol 2003;56:80–85. [PMC free article] [PubMed]
2. Bork P. The modular architecture of a new family of growth regulators related to connective tissue growth factor. FEBS Lett 1993;327:125–30. [PubMed]
3. Brigstock DR. The connective tissue growth factor/cysteine-rich 61/nephroblastoma overexpressed (CCN) family. Endocr Rev 1999;20:189–206. [PubMed]
4. Lau LF, Lam SC-T. The CCN family of angiogenic regulators: the integrin connection. Exp Cell Res 1999;248:44–57. [PubMed]
5. Perbal B. NOV (nephroblastoma overexpressed) and the CCN family of genes: structural and functional issues. Mol Pathol 2001;54:57–79. [PMC free article] [PubMed]
6. O’Brien TP, Yang GP, Sanders L, et al. Expression of cyr61, a growth factor-inducible immediate early gene. Mol Cell Biol 1990;10:3569–77. [PMC free article] [PubMed]
7. Bradham DM, Igarashi A, Potter RL, et al. Connective tissue growth factor: a cysteine-rich mitogen secreted by human vascular endothelial cells is related to the SRC-induced immediate early gene product CEF-10. J Cell Biol 1991;114:1285–94. [PMC free article] [PubMed]
8. Joliot V, Martinerie C, Dambrine G, et al. Proviral rearrangements and overexpression of a new cellular gene (nov) in myeloblastosis-associated virus type 1-induced nephroblastomas. Mol Cell Biol 1992;12:10–21. [PMC free article] [PubMed]
9. Pennica D, Swanson TA, Welsh JW, et al. WISP genes are members of the connective tissue growth factor family that are up-regulated in wnt-1-transformed cells and aberrantly expressed in human colon tumors. Proc Natl Acad Sci U S A 1998;95:14717–22. [PubMed]

Articles from Molecular Pathology : MP are provided here courtesy of BMJ Publishing Group