General Notes
Mouse anti Human Complement Factor H antibody, clone OX-23 recognizes intact human serum complement protein factor H, also known as H factor 1. Complement factor H is a 1213 amino acid ~155 kDa secreted glycoprotein bearing multiple disulphide bonds and is involved in the deactivation of C3b and dissociation of C3 convertase in the alternative complement pathway. Mouse anti Human Complement Factor H antibody, clone OX-23 also recognizes a ~43-49 kDa truncated form of factor H present at low level (1-5ug/ml) in plasma and urine.Mutations in the CFH gene can lead to the development of Complement Factor H deficiency (CFHD) which can be asymptomatic, present with recurrent bacterial infections or renal failure (Ault et al. 1997). Mutations can also lead to development of Basal laminar drusen (BLD), the deposition of extracellular deposits accumulating below the retinal pigment epithelium on Bruch membrane which can ultimately lead to vision loss (Boon et al. 2008). Additionally, mutations in the CFH gene can lead to increased susceptibility to Hemolytic uremic syndrome atypical 1 (AHUS1) or Macular degeneration, age-related, 4 (ARMD4).Mouse anti Human Complement Factor H antibody, clone OX-23 has been used successfully for the determination of levels of bound murine factor H in a sandwich ELISA (Daniels-Treffandier et al. 2016).