Immunology Antibodies 1

Anti-CFB Antibody (CAB13243)

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SKU:
CAB13243
Product Type:
Antibody
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Immunology

Description

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Antibody Name:Anti-CFB Antibody
Antibody SKU:CAB13243
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 80-420 of human CFB (NP_001701.2).
Application:WB
Recommended Dilution:WB 1:1000 - 1:2000
Reactivity:Mouse, Rat
Positive Samples:Rat serum
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 80-420 of human CFB (NP_001701.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:GSWS TLKT QDQK TVRK AECR AIHC PRPH DFEN GEYW PRSP YYNV SDEI SFHC YDGY TLRG SANR TCQV NGRW SGQT AICD NGAG YCSN PGIP IGTR KVGS QYRL EDSV TYHC SRGL TLRG SQRR TCQE GGSW SGTE PSCQ DSFM YDTP QEVA EAFL SSLT ETIE GVDA EDGH GPGE QQKR KIVL DPSG SMNI YLVL DGSD SIGA SNFT GAKK CLVN LIEK VASY GVKP RYGL VTYA TYPK IWVK VSEA DSSN ADWV TKQL NEIN YEDH KLKS GTNT KKAL QAVY SMMS WPDD VPPE GWNR TRHV IILM TDGL HNMG GDPI TVID EIRD LLYI GKDR KNPR E
Gene ID:629
Uniprot:P00751
Cellular Location:Secreted
Calculated MW:68kDa/85kDa
Observed MW:100kDa
Synonyms:CFB, AHUS4, ARMD14, BF, BFD, CFAB, CFBD, FB, FBI12, GBG, H2-Bf, PBF2
Background: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.
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
Anti-CFB Antibody (CAB13243)Western blot analysis of extracts of rat serum, using CFB antibody (CAB13243) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 10s.
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