CFI Polyclonal Antibody | MyBioSource MBS2541290 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

CFI Polyclonal Antibody

catalog :

MBS2541290

quantity :

0.06 mL

price :

150 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse

application :

western blot, immunocytochemistry

more info or order :

MyBioSource product webpage

image

image 1 :

Western blot analysis of extracts of various cells lines, using CFI Polyclonal Antibody at dilution of 1:1000

image 2 :

Immunofluorescence analysis of HeLa cell using CFI antibody. Blue: DAPI for nuclear staining.

product information

catalog number :

MBS2541290

products type :

Antibody

products full name :

CFI Polyclonal Antibody

products short name :

[CFI]

products name syn :

[FI; IF; KAF; AHUS3; ARMD13; C3BINA; C3b-INA]

other names :

[complement factor I isoform 3 preproprotein; Complement factor I; complement factor I; complement factor I; C3B/C4B inactivator]

products gene name :

[CFI]

other gene names :

[CFI; CFI; FI; IF; KAF; AHUS3; ARMD13; C3BINA; C3b-INA; IF]

uniprot entry name :

CFAI_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Rabbit

reactivity :

Human,mouse

sequence length :

576

purity :

Affinity Purification

storage stability :

Store at -20°C. Avoid freeze/thaw cycles.

tested application :

Western Blot (WB),Immunofluorescence (IF)

app notes :

WB: 1:500 - 1:2000. IF: 1:50 - 1:100

image1 heading :

Western Blot (WB)

image2 heading :

Immunofluorescence (IF)

other info1 :

Immunogen: Recombinant protein of human CFI. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. Calculated MW: 66 kDa. Observed MW: 70 kDa

ncbi gi num :

1061899980

ncbi gb acc num :

NM_001331035.1

ncbi pathways :

Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Complement Cascade Pathway (1269241); Immune System Pathway (1269170); Innate Immune System Pathway (1269203); Regulation Of Complement Cascade Pathway (1269250); Staphylococcus Aureus Infection Pathway (172846); Staphylococcus Aureus Infection Pathway (171867)

ncbi summary :

This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uremic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immune deposits and age-related macular degeneration are other conditions associated with mutations of this gene. [provided by RefSeq, Dec 2015]

uniprot summary :

CFI: Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively. Defects in CFI are a cause of susceptibility to hemolytic uremic syndrome atypical type 3 (AHUS3). 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. Defects in CFI are the cause of complement factor I deficiency (CFI deficiency). CFI deficiency is an autosomal recessive condition associated with a propensity to pyogenic infections. Belongs to the peptidase S1 family. Protein type: EC 3.4.21.45; Protease; Secreted; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 4q25. Cellular Component: extracellular region; extracellular space. Molecular Function: protein binding; serine-type endopeptidase activity. Biological Process: regulation of complement activation. Disease: Complement Factor I Deficiency; Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 3; Macular Degeneration, Age-related, 13

size1 :

0.06 mL

price1 :

150 USD

size2 :

0.12 mL

price2 :

225

size3 :

0.2 mL

price3 :

360

more info or order :

MyBioSource product webpage