protein

Complement Factor B

MBS537119

0.25 mg

485 USD

Native Protein

Complement Factor B

Complement Factor B

Recombinant CFB

factor B; Complement factor B; complement factor B; complement factor B; C3/C5 convertase; Glycine-rich beta glycoprotein; GBG

CFB; CFB; BF; FB; BFD; GBG; CFAB; CFBD; PBF2; AHUS4; FBI12; H2-Bf; ARMD14; BF; BFD; GBG

CFAB_HUMAN

Human

621

> 98% by SDS-PAGE

Supplied as a liquid in PBS, pH 7.2, no preservatives added,

1.05 mg/ml

Upon receipt, store at -70 degree C. Avoid repeated freeze/thaw cycles. Shipping: Ships with Dry Ice

User optimized

Source Note: Human serum/plasma. Protein Type: Native. Biological Significance: Complement factor B is a glycosylated protein composed of a single 93,000 Da polypeptide chain. It is an essential component of the alternative pathway of complement activation and is found in plasma at approximately 200 ug/mL. In the presence of Mg++ factor B binds to C3b and the C3b,B complex can be activated by factor D, a serine protease that circulates as an active trypsin-like serine protease. Bioactivity: 4, 700 U/mg - 100 % of factor B activity in NHS on a mg/mg basis

Biohazard Information: Donor samples were tested and found to be negative for HBsAg, HTLV-I/II, STS, and for antibodies to HCV, HIV-1 and HIV-II. Nonetheless caution should be used when handling this material as there is a margin of error in all tests. Dry Ice Shipment: Extra charge fee may add to your shipping cost as dry ice is required to ship this product. Grade: Purified. Contaminants: < trace amounts of IgG, IgA, IgM, albumin, C2, C3, C4, Factor H, or Factor 1. Biohazard: Human serum/plasma starting material was tested and found negative for Anti-HIV-I/II antibodies, Anti-HCV Ab, HBsAg, HTLV-i/II and STS by FDA approved methods.

Immunology; Native Protein

Purified native Human Complement Factor B

13560705

AAK30167.1

P00751

93 kDa

Activation Of C3 And C5 Pathway (106412); Alternative Complement Activation Pathway (106410); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Complement Cascade Pathway (106405); Immune System Pathway (106386); Initial Triggering Of Complement Pathway (106406); Innate Immune System Pathway (106387); Regulation Of Complement Cascade Pathway (576254); Staphylococcus Aureus Infection Pathway (172846)

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: Protease; Secreted, signal peptide; EC 3.4.21.47; Secreted. Chromosomal Location of Human Ortholog: 6p21.3. Cellular Component: extracellular space; extracellular region; plasma membrane. 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

0.25 mg

485 USD