Complement Factor Bb Mouse anti-Human, Clone: 10-09, Invitrogen
Mouse Monoclonal Antibody
Manufacturer: Invitrogen MA528085
This antibody recognizes the Bb fragment of 90 kDa human complement factor B, present in blood serum. C3b associates with complement factor B, inducing conformational change which enables complement factor D to cleave the N-terminal of complement factor B, the Ba subunit, leaving the 63 kDa Bb subunit associated with C3b and forming C3 convertase. Subunit Bb is involved in the proliferation of preactivated B lymphocytes. Conversely, Ba inhibits lymphocyte proliferation.CFB is involved in hemolytic uremic syndrome atypical 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. Disease susceptibility is associated with variations affecting the gene represented in this entry. 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.
|Complement Factor Bb|
|BBS with 0.1% sodium azide|
|Human factor B|
|ELISA, Flow Cytometry, Immunohistochemistry (Frozen), Western Blot|
|4° C, do not freeze|
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