Human CFB (26-259) Recombinant Protein (RPPB3155)

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SKU:
RPPB3155
Product type:
Recombinant Protein
Size:
20ug
Species:
Human
Target:
CFB (26-259)
Synonyms:
Complement factor B (EC:342147)
Synonyms:
C3/C5 convertase
Synonyms:
Glycine-rich beta glycoprotein
Synonyms:
GBG
Source:
Escherichia Coli
Uniprot:
P00751
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Description

Product Name:Human CFB (26-259) Recombinant Protein
Product Code:RPPB3155
Size:20µg
Species:Human
Target:CFB (26-259)
Synonyms:Complement factor B (EC:3.4.21.47), C3/C5 convertase, Glycine-rich beta glycoprotein, GBG, PBF2, Properdin factor B, Complement factor B Ba fragment, Complement factor B Bb fragment, CFB, Complement Factor B, BFD, AHUS4, BF, BFD, CFAB, FB, FBI12, H2-Bf.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:The CFB solution (1mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 10% glycerol and 0.4M Urea.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 85.0% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSQ SHMTPWSLAR PQGSCSLEGV EIKGGSFRLL QEGQALEYVC PSGFYPYPVQ TRTCRSTGSW STLKTQDQKT VRKAECRAIH CPRPHDFENG EYWPRSPYYN VSDEISFHCY DGYTLRGSAN RTCQVNGRWS GQTAICDNGA GYCSNPGIPI GTRKVGSQYR LEDSVTYHCS RGLTLRGSQR RTCQEGGSWS GTEPSCQDSF MYDTPQEVAE AFLSSLTETI EGVDAEDGHG PGEQQKR

Complement Factor B, also known as CFB, encodes complement factor B which is a component of the alternative pathway of complement activation. Factor B circulates in the blood as a single chain polypeptide. Once the alternative pathway is activated 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 connects with C3b to form the alternative pathway C3 convertase. Also, Bb is involved in the proliferation of preactivated B lymphocytes, while Ba inhibits their proliferation.

CFB (26-259) Human Recombinant produced in E. coli is. a single polypeptide chain containing 257 amino acids and having a molecular mass of 28.4kDa. CFB is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

UniProt Protein Function: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.
UniProt Protein Details:

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

Chromosomal Location of Human Ortholog: 6p21.3

Cellular Component: extracellular region; extracellular space; plasma membrane

Molecular Function:complement binding; serine-type endopeptidase activity

Biological Process: complement activation; complement activation, alternative pathway; regulation of complement activation

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

NCBI Summary: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 Code:P00751
NCBI GenInfo Identifier:584908
NCBI Gene ID:629
NCBI Accession:P00751.2
UniProt Secondary Accession:P00751,O15006, Q29944, Q53F89, Q5JP67, Q5ST50, Q96HX6 Q9BTF5, Q9BX92, B0QZQ6,
UniProt Related Accession:P00751
Molecular Weight:68,872 Da
NCBI Full Name:Complement factor B
NCBI 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
UniProt Synonym Protein Names:C3/C5 convertase; Glycine-rich beta glycoprotein; GBG
Protein Family:Complement factor
UniProt Gene Name:CFB  
UniProt Entry Name:CFAB_HUMAN
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