Alternative titles; symbols
HGNC Approved Gene Symbol: CCL13
Cytogenetic location: 17q12 Genomic coordinates (GRCh38): 17:34,356,480-34,358,610 (from NCBI)
Chemokines are structurally and functionally related 8- to 10-kD polypeptides involved in recruitment of white blood cells to areas of inflammation and their subsequent activation. In addition, some chemokines regulate the proliferative potential of hematopoietic progenitor cells, endothelial cells, and some types of transformed cells. Chemokines can be divided into CXC and CC families based on whether their first 2 cysteines are separated by 1 amino acid or are adjacent, respectively. CXC chemokines preferentially attract and affect neutrophils, whereas CC chemokines chemoattract and affect eosinophils, monocytes, and T cells. CCL13 belongs to the CC chemokine family (summary by Berkhout et al., 1997).
By EST database searching and PCR, Berkhout et al. (1997) obtained a cDNA encoding CCL13, which they called MCP4. The deduced 98-amino acid MCP4 protein has a calculated molecular mass of 8.6 kD and shares 62% and 61% similarity with MCP1 (CCL2; 158105) and MCP3 (CCL7; 158106), respectively. Immunoblot analysis showed expression of a 9-kD protein. Northern blot analysis revealed expression of a 1.0-kb transcript in human small intestine, colon, thymus, lung, trachea, stomach, lymph node, and colonic mucosa, but not in various other tissues. Immunohistochemical analysis demonstrated expression in luminal endothelium of human atherosclerotic coronary vessels and in macrophage-rich areas of carotid plaques.
Naruse et al. (1996) identified on chromosome 17q11.2 a cluster of chemokines from the CC family. They mapped 1 gene of the CC family, called NCC1 by them, to the cluster using a panel of somatic cell hybrids with known deletions. NCC1 was originally characterized as an expressed sequence tag (EST). The deduced order of genes from a YAC contig assembled for the region is cen--NF1 (613113)--MCP3 (158106), MCP1 (158105), NCC1, I-309 (182281)--Y1741 breakpoint--RANTES (187011)--LD78-gamma, AT744.2 (603782), LD78-beta (601395)--NCC3 (601393), NCC2 (601392), AT744.1 (182284), LD78-alpha (182283)--NCC4 (601394)--RARA (180240)--tel.
Berkhout et al. (1997) found that human MCP4 attracted monocytes and induced increased cytosolic Ca(2+) concentrations. Binding analysis showed that MCP4 interacted with CKR2B (601267).
Minimal change nephrotic syndrome (MCNS) is defined by selective massive proteinuria and a relapsing/remitting course without histologic evidence of classic immune mechanism-mediated injury. Komatsuda et al. (2008) noted that it had been hypothesized that MCNS may result from immune cell dysfunction leading to release of glomerular permeability factors. Using cDNA microarray analysis, Komatsuda et al. (2008) identified 171 functional genes, including CCL13 and HSPC159 (LGALSL; 617902), that were upregulated at least 2-fold in peripheral blood mononuclear cells (PBMCs) from 2 MCNS patients during the nephrosis phase compared with the remission phase. Quantitative RT-PCR showed that expression of CCL13 and HSPC159 mRNA was higher in nephrotic PBMC samples than in remission PBMC samples from all 24 MCNS patients examined, whereas these mRNA expression patterns were variable among 10 patients with membranous nephropathy (MN). CCL13 and HSPC159 mRNA expression was significantly higher in PBMCs from nephrotic MCNS patients than in PBMCs from nephrotic MN patients and healthy controls. Komatsuda et al. (2008) concluded that CCL13 and HSPC159 are upregulated specifically during the nephrotic phase in MCNS patients.
Berkhout, T. A., Sarau, H. M., Moores, K., White, J. R., Elshourbagy, N., Appelbaum, E., Reape, T. J., Brawner, M., Makwana, J., Foley, J. J., Schmidt, D. B., Imburgia, C., McNulty, D., Matthews, J., O'Donnell, K., O'Shannessy, D., Scott, M., Groot, P. H. E., Macphee, C. Cloning, in vitro expression, and functional characterization of a novel human CC chemokine of the monocyte chemotactic protein (MCP) family (MCP-4) that binds and signals through the CC chemokine receptor 2B. J. Biol. Chem. 272: 16404-16413, 1997. [PubMed: 9195948] [Full Text: https://doi.org/10.1074/jbc.272.26.16404]
Komatsuda, A., Wakui, H., Iwamoto, K., Harada, M., Okumoto, Y., Sawada, K. Gene expression profiling of peripheral blood mononuclear cells from patients with minimal change nephrotic syndrome by cDNA microarrays. Am. J. Nephrol. 28: 539-547, 2008. [PubMed: 18219197] [Full Text: https://doi.org/10.1159/000114098]
Naruse, K., Ueno, M., Satoh, T., Nomiyama, H., Tei, H., Takeda, M., Ledbetter, D. H., Van Coillie, E., Opdenakker, G., Gunge, N., Sakaki, Y., Iio, M., Miura, R. A YAC contig of the human CC chemokine genes clustered on chromosome 17q11.2. Genomics 34: 236-240, 1996. [PubMed: 8661057] [Full Text: https://doi.org/10.1006/geno.1996.0274]