Anti-Factor H/Cfh Antibody Picoband® Fluoro594 Conjugated

Factor H is a member of the regulators of complement activation family and is a complement control protein. This gene is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short consensus repeat (SCR) domains. This protein is secreted into the bloodstream and has an essential role in the regulation of complement activation, restricting this innate defense mechanism to microbial infections. Mutations in this gene have been associated with hemolytic-uremic syndrome (HUS) and chronic hypocomplementemic nephropathy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.

Metalloproteinase inhibitor 1; Erythroid-potentiating activity; EPA; Fibroblast collagenase inhibitor; Collagenase inhibitor; Tissue inhibitor of metalloproteinases 1; TIMP-1; TIMP1; CLGI, TIMP

Angiogenesis, Cancer, Cardiovascular, Cell Biology, Cytoskeleton/ECM, ECM Enzymes, Extracellular Matrix, Invasion/Microenvironment, Protease Inhibitors, Proteolysis/Ubiquitin, Signal Transduction

Metalloproteinase inhibitor that functions by forming one to one complexes with target metalloproteinases, such as collagenases, and irreversibly inactivates them by binding to their catalytic zinc cofactor. Acts on MMP1, MMP2, MMP3, MMP7, MMP8, MMP9, MMP10, MMP11, MMP12, MMP13 and MMP16. Does not act on MMP14. Also functions as a growth factor that regulates cell differentiation, migration and cell death and activates cellular signaling cascades via CD63 and ITGB1. Plays a role in integrin signaling. Mediates erythropoiesis in vitro; but, unlike IL3, it is species-specific, stimulating the growth and differentiation of only human and murine erythroid progenitors.

1. Abrera-Abeleda, M. A., Nishimura, C., Smith, J. L. H., Sethi, S., McRae, J. L., Murphy, B. F., Silvestri, G., Skerka, C., Jozsi, M., Zipfel, P. F., Hageman, G. S., Smith, R. J. H. Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with membranoproliferative glomerulonephritis type II (dense deposit disease) . J. Med. Genet. 43: 582-589, 2006. 2. Arakawa, S., Takahashi, A., Ashikawa, K., Hosono, N., Aoi, T., Yasuda, M., Oshima, Y., Yoshida, S., Enaida, H., Tsuchihashi, T., Mori, K., Honda, S., Negi, A., Arakawa, A., Kadonosono, K., Kiyohara, Y., Kamatani, N., Nakamura, Y., Ishibashi, T., Kubo, M. Genome-wide association study identifies two susceptibility loci for exudative age-related macular degeneration in the Japanese population. Nature Genet. 43: 1001-1004, 2011. 3. Ault, B. H., Schmidt, B. Z., Fowler, N. L., Kashtan, C. E., Ahmed, A. E., Vogt, B. A., Colten, H. R. Human factor H deficiency: mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism. J. Biol. Chem. 272: 25168-25175, 1997.