Rabbit anti-Human CFB Polyclonal Antibody | anti-CFB antibody

CFB, ID (Complement Factor B, C3/C5 Convertase, Properdin Factor B, Glycine-rich beta Glycoprotein, GBG, PBF2, Complement factor B Ba fragment, Complement factor B Bb fragment, BFD, BF) (MaxLight 490)

Cat. Num. AAA6469997

• Gene Names: CFB; BF; FB; BFD; GBG; CFAB; CFBD; PBF2; AHUS4; FBI12; H2-Bf; ARMD14
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Ammonium Sulfate Precipitation.
• Synonyms: CFB; Polyclonal Antibody; ID (Complement Factor B; C3/C5 Convertase; Properdin Factor B; Glycine-rich beta Glycoprotein; GBG; PBF2; Complement factor B Ba fragment; Complement factor B Bb fragment; BFD; BF) (MaxLight 490); anti-CFB antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human CFB.
• Purity/Purification: Purified by Ammonium Sulfate Precipitation.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight490.

• Applicable Applications for anti-CFB antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:50-1:100; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the center region of human BF (KLH).
• Conjugate: MaxLight490
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight490 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western blot analysis of BF Antibody (Center) in HepG2 cell line and mouse liver tissue lysates (35ug/lane). BF (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(BF Antibody (Center) (RB18685) IHC analysis in formalin fixed and paraffin embedded human hepatocarcinoma tissue followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the BF Antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.)

Product Categories/Family for anti-CFB antibody

Antibodies; Complement

References

1. Baatz, H., Klin Monatsbl Augenheilkd 226(8), 654-658 (2009). 2. Goring, K., Am. J. Physiol., Cell Physiol. 296(5), C1140-C1150 (2009).

NCBI and Uniprot Product Information

• NCBI GI #: 67782358
• NCBI GeneID: 629
• NCBI Accession #: NP_001701
• NCBI GenBank Nucleotide #: NM_001710.5
• UniProt Accession #: P00751; O15006; Q29944; Q53F89; Q5JP67; Q5ST50; Q96HX6; Q9BTF5; Q9BX92; B0QZQ6
• Molecular Weight: 59.4kDa (528aa)
• NCBI Official Full Name: complement factor B preproprotein
• NCBI Official Synonym Full Names: complement factor B
• NCBI Official Symbol: CFB
• NCBI Official Synonym Symbols: BF; FB; BFD; GBG; CFAB; CFBD; PBF2; AHUS4; FBI12; H2-Bf; ARMD14
• NCBI Protein Information: complement factor B
• UniProt Protein Name: Complement factor B
• Protein Family: Complement factor
• UniProt Gene Name: CFB
• UniProt Synonym Gene Names: BF; BFD; GBG
• UniProt Entry Name: CFAB_HUMAN

NCBI Description

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]

Uniprot Description

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, signal peptide; Secreted; Protease; EC 3.4.21.47

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