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

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Catalog # MBS315983
Unit / Price
  1 mL  /  $435 +1 FREE 8GB USB
anti-CFH antibody
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
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Chromosome Location
Chromosome: 1; NC_000001.10 (196621008..196716634). Location: 1q32
OMIM
126700
3D Structure
ModBase 3D Structure for P08603
Clonality
Polyclonal
Host
Sheep
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)
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.
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.
NCBI/Uniprot data below describe general gene information for CFH. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
139,096 Da[Similar Products]
NCBI Official Full Name
factor H
NCBI Official Synonym Full Names
complement factor H
NCBI Official Symbol
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
UniProt Gene Name
UniProt Synonym Gene Names
HF; HF1; HF2  [Similar Products]
UniProt Entry Name
CFAH_HUMAN
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]
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.
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

Precautions
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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.

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