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anti-CFH antibody :: Sheep Factor H (Beta 1H) Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS315983 anti-CFH antibody
Unit / Price
1 mL  /  $415 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Factor H (Beta 1H) (CFH), Polyclonal Antibody

 Also Known As   

Sheep anti Factor H (Beta 1H)

 Product Synonym Names    Sheep Antibody to Human Factor H (Beta 1H)
 Product Gene Name   

anti-CFH antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Chromosome Location    Chromosome: 1; NC_000001.10 (196621008..196716634). Location: 1q32
 OMIM    126700
 3D Structure    ModBase 3D Structure for P08603
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 Clonality    Polyclonal
 Host    Sheep
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 Specificity    Factor H (Beta 1H)
Gives a single arc when tested by IEP against human plasma. Identity is confirmed by double diffusion (Ouchterlony) against human plasma and a known anti-human Factor H. The cross reactivity of this product against the sera of the sp
 Purity/Purification    Delipidated and if necessary, absorbed to monospecificity by use of solid-phase absorbants. An immunoglobulin fraction is then produced. The titer is adjusted so that inter-batch variation is within 10%.
Product is 0.2um filtered.
 Form/Format    Purified, Liquid
 Concentration    9.5mg/ml (OD280nm, E^1% = 14.5) (lot specific)
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 Immunogen    Human 155 kDa Factor H purified from plasma, shown to be pure by IEP
 Buffer    Glycine buffered saline, pH 7.4
 Preservative    0.1% Sodium azide, 0.1% EACA, 0.01% Benzamidine, 1mM EDTA
 Important Note    Centrifuge before opening to ensure complete recovery of vial contents.
 Warnings    This product contains sodium azide, which has been classified as Xn (Harmful), in European Directive 67/548/EEC in the concentration range of 0.1 - 1.0 %. When disposing of this reagent through lead or copper plumbing, flush with copious volumes of water to prevent azide build-up in drains.
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 Preparation and Storage    Upon receipt, store at 2 to 8 degree C. Slight precipitation can occur on storage, which may be removed by centrifugation and should not affect performance characteristics.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 Other Notes    Small volumes of anti-CFH antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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 Product Categories/Family for anti-CFH antibody    Polyclonal Antibodies to Platelets and Hemostasis Antigens
NCBI/Uniprot data below describe general gene information for CFH. It may not necessarily be applicable to this product.
 NCBI GI #    31965
 NCBI GeneID    3075
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 UniProt Primary Accession #    P08603 [Other Products]
 UniProt Secondary Accession #    P78435; Q14570; Q2TAZ5; Q38G77; Q5TFM3; Q8N708; Q9NU86; A5PL14 [Other Products]
 UniProt Related Accession #    P08603; Q14006; Q5TFM2 [Other Products]
 Molecular Weight    139,096 Da [Similar Products]
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 NCBI Official Full Name    factor H
 NCBI Official Synonym Full Names    complement factor H
 NCBI Official Symbol    CFH [Similar Products]
 NCBI Official Synonym Symbols   
FH; HF; HF1; HF2; HUS; FHL1; AHUS1; AMBP1; ARMD4; ARMS1; CFHL3; MGC88246
[Similar Products]
 NCBI Protein Information    complement factor H; beta-1H; factor H-like 1; beta-1-H-globulin; OTTHUMP00000033598; OTTHUMP00000034106; H factor 1 (complement); H factor 2 (complement); adrenomedullin binding protein; complement factor H, isoform b; age-related maculopathy susceptibility 1
 UniProt Protein Name    Complement factor H
 UniProt Synonym Protein Names   
H factor 1
 Protein Family    Complement factor
 UniProt Gene Name    CFH [Similar Products]
 UniProt Synonym Gene Names    HF; HF1; HF2 [Similar Products]
 UniProt Entry Name    CFAH_HUMAN
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 NCBI Summary for CFH    This gene is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short concensus repeat (SCR) domains. This protein is secreted into the bloodstream and has an essential role in the regulation of complement activation, restricting this innate defense mechanism to microbial infections. Mutations in this gene have been associated with hemolytic-uremic syndrome (HUS) and chronic hypocomplementemic nephropathy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. [provided by RefSeq]
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 UniProt Comments for CFH    Function: Factor H functions as a cofactor in the inactivation of C3b by factor I and also increases the rate of dissociation of the C3bBb complex (C3 convertase) and the (C3b)NBB complex (C5 convertase) in the alternative complement pathway.

Subcellular location: Secreted.

Tissue specificity: Expressed by the liver and secreted in plasma.

Involvement in disease: Genetic variations in CFH are associated with basal laminar drusen (BLD) [

MIM:126700]; also known as drusen of Bruch membrane or cuticular drusen or grouped early adult-onset drusen. Drusen are extracellular deposits that accumulate below the retinal pigment epithelium on Bruch membrane. Basal laminar drusen refers to an early adult-onset drusen phenotype that shows a pattern of uniform small, slightly raised yellow subretinal nodules randomly scattered in the macula. In later stages, these drusen often become more numerous, with clustered groups of drusen scattered throughout the retina. In time these small basal laminar drusen may expand and ultimately lead to a serous pigment epithelial detachment of the macula that may result in vision loss.Defects in CFH are the cause of complement factor H deficiency (CFH deficiency) [

MIM:609814]. CFH deficiency determines uncontrolled activation of the alternative complement pathway with consumption of C3 and often other terminal complement components. It is associated with a number of renal diseases with variable clinical presentation and progression, including membranoproliferative glomerulonephritis and atypical hemolytic uremic syndrome. CFH deficiency patients may show increased susceptibility to meningococcal infections.Defects in CFH are a cause of susceptibility to hemolytic uremic syndrome atypical type 1 (AHUS1) [

MIM:235400]. 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. Note=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. Ref.19 Ref.20 Ref.21 Ref.26 Ref.28 Ref.29 Ref.30 Ref.38Genetic variation in CFH is associated with age-related macular degeneration type 4 (ARMD4) [

MIM:610698]. ARMD is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid (known as drusen) that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane.

Sequence similarities: Contains 20 Sushi (CCP/SCR) domains.

Sequence caution: The sequence CAB41739.1 differs from that shown. Reason: Frameshift at position 341.
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Product References and Citations for anti-CFH antibody

   • Chung, Kyung Min., et al., (2006), "West Nile virus nonstructural protein NS1 inhibits complement activation by binding the regulatory protein Factor H", Proc. National Academy Science, USA, 103 (50), 19111-19116
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 Research Articles on CFH    1. Zinc binds weakly to complement factor H at multiple surface locations (most probably within the functionally important SCR-6/8 domains), and this explains why zinc inhibits complement factor H activity.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-CFH antibodyPathways associated with anti-CFH antibody
 Reference Product  PubMed Publications
 CFHR1 antibody  >35 publications with CFH and CFHR1
 CFI antibody  >34 publications with CFH and CFI
 CRP antibody  >16 publications with CFH and CRP
 ITGAM antibody  >3 publications with CFH and ITGAM
 Products by Pathway  Pathway Diagram
 Complement And Coagulation Cascades Pathway antibodies  Complement And Coagulation Cascades Pathway Diagram
 Complement And Coagulation Cascades Pathway antibodies  Complement And Coagulation Cascades Pathway Diagram
 Staphylococcus Aureus Infection Pathway antibodies  Staphylococcus Aureus Infection Pathway Diagram
 Staphylococcus Aureus Infection Pathway antibodies  Staphylococcus Aureus Infection Pathway Diagram
Diseases associated with anti-CFH antibodyOrgans/Tissues associated with anti-CFH antibody
 Disease Name  Pubmed Publications
 Macular Degeneration Antibodies  >268 publications with CFH and Macular Degeneration
 Retinal Degeneration Antibodies  >228 publications with CFH and Retinal Degeneration
 Female Urogenital Diseases Antibodies  >86 publications with CFH and Female Urogenital Diseases
 Kidney Diseases Antibodies  >84 publications with CFH and Kidney Diseases
 Anemia Antibodies  >71 publications with CFH and Anemia
 Atypical hemolytic uremic syndrome Antibodies  >54 publications with CFH and Atypical hemolytic uremic syndrome
 Inflammation Antibodies  >45 publications with CFH and Inflammation
 COMPLEMENT FACTOR H DEFICIENCY Antibodies  >40 publications with CFH and COMPLEMENT FACTOR H DEFICIENCY
 Cardiovascular Diseases Antibodies  >40 publications with CFH and Cardiovascular Diseases
 Neovascularization, Pathologic Antibodies  >36 publications with CFH and Neovascularization, Pathologic
 Organ/Tissue Name  Pubmed Publications
 Eye Antibodies  >122 publications with CFH and Eye
 Kidney Antibodies  >56 publications with CFH and Kidney
 Vascular Antibodies  >33 publications with CFH and Vascular
 Ear Antibodies  >31 publications with CFH and Ear
 Liver Antibodies  >21 publications with CFH and Liver
 Brain Antibodies  >20 publications with CFH and Brain
 Heart Antibodies  >17 publications with CFH and Heart
 Nerve Antibodies  >9 publications with CFH and Nerve
 Skin Antibodies  >8 publications with CFH and Skin
 Embryonic Tissue Antibodies  >5 publications with CFH and Embryonic Tissue
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