Complement Factor B, ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 6; NC_000006.11 (31913721..31919861). Location: 6p21.3

Store all reagents at 2-8 degree C

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Small volumes of CFB elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS023074 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Complement Factor B (CFB) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing CFB. The ELISA analytical biochemical technique of the MBS023074 kit is based on CFB antibody-CFB antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect CFB antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, CFB. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

85,533 Da

complement factor B

complement factor B; C3 proactivator; C3/C5 convertase; C3 proaccelerator; properdin factor B; B-factor, properdin; glycine-rich beta glycoprotein; glycine-rich beta-glycoprotein

Complement factor B

BF; BFD; GBG  [Similar Products]

CFAB_HUMAN

This gene encodes complement factor B, a component of the alternative pathway of complement activation. Factor B circulates in the blood as a single chain polypeptide. Upon activation of the alternative pathway, it is cleaved by complement factor D yielding the noncatalytic chain Ba and the catalytic subunit Bb. The active subunit Bb is a serine protease which associates with C3b to form the alternative pathway C3 convertase. Bb is involved in the proliferation of preactivated B lymphocytes, while Ba inhibits their proliferation. This gene localizes to the major histocompatibility complex (MHC) class III region on chromosome 6. This cluster includes several genes involved in regulation of the immune reaction. Polymorphisms in this gene are associated with a reduced risk of age-related macular degeneration. The polyadenylation site of this gene is 421 bp from the 5' end of the gene for complement component 2. [provided by RefSeq, Jul 2008]

CFB: Factor B which is part of the alternate pathway of the complement system is cleaved by factor D into 2 fragments: Ba and Bb. Bb, a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase. It has also been implicated in proliferation and differentiation of preactivated B- lymphocytes, rapid spreading of peripheral blood monocytes, stimulation of lymphocyte blastogenesis and lysis of erythrocytes. Ba inhibits the proliferation of preactivated B-lymphocytes. Defects in CFB are a cause of susceptibility to hemolytic uremic syndrome atypical type 4 (AHUS4). An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype. Belongs to the peptidase S1 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted; Protease; Secreted, signal peptide; EC 3.4.21.47

Chromosomal Location of Human Ortholog: 6p21.3

Cellular Component: extracellular space; plasma membrane; extracellular region

Molecular Function: complement binding; serine-type endopeptidase activity

Biological Process: complement activation, alternative pathway; regulation of complement activation; innate immune response; proteolysis; complement activation

Disease: Macular Degeneration, Age-related, 14; Complement Factor B Deficiency; Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 4

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