|Other Names||Complement factor H, H factor 1, CFH, HF, HF1, HF2|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Synonyms||HF, HF1, HF2|
|Function||Factor H functions as a cofactor in the inactivation of C3b by factor I and also increases the rate of dissociation of the C3bBb complex (C3 convertase) and the (C3b)NBB complex (C5 convertase) in the alternative complement pathway.|
|Tissue Location||Expressed by the liver and secreted in plasma.|
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This gene is a member of the Regulator of ComplementActivation (RCA) gene cluster and encodes a protein with twentyshort concensus repeat (SCR) domains. This protein is secreted intothe bloodstream and has an essential role in the regulation ofcomplement activation, restricting this innate defense mechanism tomicrobial infections. Mutations in this gene have been associatedwith hemolytic-uremic syndrome (HUS) and chronic hypocomplementemicnephropathy. Alternate transcriptional splice variants, encodingdifferent isoforms, have been characterized.
Dieterich, R., et al. Infect. Immun. 78(11):4467-4476(2010)Sofat, R., et al. Atherosclerosis 213(1):184-190(2010)Davila, S., et al. Nat. Genet. 42(9):772-776(2010)Scambi, C., et al. PLoS ONE 5 (8), E12162 (2010) :Bunkenborg, J., et al. Proteomics 4(2):454-465(2004)
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