Complement Factor B Recombinant Protein | CFB recombinant protein

Recombinant Complement Factor B

Cat. Num. AAA201168

• Gene Names: CFB; BF; FB; BFD; GBG; CFAB; CFBD; PBF2; AHUS4; FBI12; H2-Bf; ARMD14
• Applications: SDS-Page
• Purity: > 90% by SDS - PAGE
• Synonyms: Complement Factor B; Recombinant Complement Factor B; CFB; C3/C5 convertase; Glycine-rich beta glycoprotein; GBG; PBF2; Properdin factor B; BF; BFD; AHUS4; ARMD14; CFAB; CFBD; FB; FBI12; H2-Bf; CFB recombinant protein

• Host: Baculovirus
• Purity/Purification: > 90% by SDS - PAGE
• Form/Format: Liquid. In Phosphate-Buffered Saline (pH 7.4) containing 20% glycerol.
• Concentration: 0.5mg/ml (determined by Absorbance at 280nm) (varies by lot)
• Sequence Positions: 260-764aa
• Sequence: KIVLDPS GSMNIYLVLD GSDSIGASNF TGAKKCLVNL IEKVASYGVK PRYGLVTYAT YPKIWVKVSE ADSSNADWVT KQLNEINYED HKLKSGTNTK KALQAVYSMM SWPDDVPPEG WNRTRHVIIL MTDGLHNMGG DPITVIDEIR DLLYIGKDRK NPREDYLDVY VFGVGPLVNQ VNINALASKK DNEQHVFKVK DMENLEDVFY QMIDESQSLS LCGMVWEHRK GTDYHKQPWQ AKISVIRPSK GHESCMGAVV SEYFVLTAAH CFTVDDKEHS IKVSVGGEKR DLEIEVVLFH PNYNINGKKE AGIPEFYDYD VALIKLKNKL KYGQTIRPIC LPCTEGTTRA LRLPPTTTCQ QQKEELLPAQ DIKALFVSEE EKKLTRKEVY IKNGDKKGSC ERDAQYAPGY DKVKDISEVV TPRFLCTGGV SPYADPNTCR GDSGGPLIVH KRSRFIQVGV ISWGVVDVCK NQKRQKQVPA HARDFHINLF QVLPWLKEKL QDEDLGFL
• Sequence Length: 764

• Applicable Applications for CFB recombinant protein: SDS-PAGE
• Tag: His-Tag
• Predicted nterm: Ala
• Endotoxin: < 1 EU per 1ug of protein (determined by LAL method)
• Preparation and Storage: Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoidrepeated freezing and thawing cycles.

SDS-Page

(3ug by SDS-PAGE under reducing condition and visualized by coomassie blue stain)

Related Product Information for CFB recombinant protein

Complement factor B, also known as CFB, is 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, itis cleaved by complement factor D yielding the noncatalytic chain Ba and the catalytic subunit Bb. The activesubunit Bb is a serine protease which associates with C3b to form the alternative pathway C3 convertase. Bb isinvolved in the proliferation of preactivated B lymphocytes, while Ba inhibits their proliferation. Recombinant human Complement factor B protein, fused to His-tag at C-terminus, was expressed in insect cell and purified byusing conventional chromatography techniques.

Product Categories/Family for CFB recombinant protein

Immunology

References

Francis PJ., et al. (2009) J Med Genet. 46:300-307.;;Schwaeble W., et al (1993). Immunobiology 188:221-232.

NCBI and Uniprot Product Information

• NCBI GI #: 67782358
• NCBI GeneID: 629
• NCBI Accession #: NP_001701.2
• NCBI GenBank Nucleotide #: NP_001701.2
• UniProt Accession #: P00751
• Molecular Weight: 58.1kDa (514aa)
• 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