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Sheep anti Factor H (Beta 1H)

ArtNr 18-511-245436
Hersteller GENWAY
Menge 0.1ml
Kategorie
Typ Antibody
Specific against other
Host Sheep
ECLASS 10.1 32160702
ECLASS 11.0 32160702
UNSPSC 12352203
Alias GWB-DBD44C
Similar products 18-511-245436
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Genway ID:
GWB-DBD44C
Host Animal:
Sheep
Immunogen:
Human 155 kDa Factor H purified from plasma shown to be pure by IEP
Specificity:
Factor H (Beta 1H)
Specificity:
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 species listed below has been assessed by double diffusion using 10ul of antisera against 10ul of species sera. These results are intended as a guideline only. It is possible that cross-reactivity against species listed as negative above may be observed when using different test conditions or techniques. Cat + Chicken - Cow - Dog + G. Pig + Horse + Human + Mouse + Pig - Rabbit - Rat + Goat -
Type of Product:
Polyclonal Antibodies to Platelets and Hemostasis Antigens
Concentration:
9. 5mg/ml (OD280nm E1% = 14. 5)Preservatives: NaN3
Buffer:
Glycine buffered saline pH 7. 4Applications Notes : Suitable for use in a variety of gel techniques including radial immunodiffusion (RID) double diffusion immunoelectrophoresis (IEP). The use of 3% PEG 6000 with 1. 2% agarose in a suitable buffer (such as TBE or tris-barbital pH > 8. 2) is recommended. Suitability for use in nephelometry enzyme-linked immunosorbent assays and Western blot and IHC has not been assessed but use in such assays should not necessarily be excluded. Each laboratory should determine an optimum working titer for use in its particular application. . RID : 1ul antiserum/cm2 gel vs. 5ul neat - 1:10 dilution human plasma. . Double Dif
Fusion:
10ul antiserum vs. 10ul plasma. . IEP: 100ul antiserum vs. 5ul plasma.
Warning:
This product contains sodium azide which has been classified as Xn (Harmful) in European Directive 67/548/EEC in the concentration range of 0. 1â ??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. Sheep anti Factor H (Beta 1H). Sheep Antibody to Human Factor H (Beta 1H)
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.
Disease:
Genetic variations in CFH are associated with basal laminar drusen [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.
Disease:
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.
Disease:
Defects in CFH are a cause of hemolytic-uremic syndrome (HUS) [MIM:235400]. HUS is a microvasculature disorder leading to microangiopathic hemolytic anemia associated with distorted erythrocytes (\' burr cells\' ) thrombocytopenia and acute renal failure. Both dominant and recessive modes of inheritance have been reported. Most cases of HUS are associated with epidemics of diarrhea caused by verocytotoxin-producing bacteria but atypical cases of HUS not associated with diarrhea (aHUS) also occur.
Disease:
Genetic 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.
Similarity:
Contains 20 Sushi (CCP/SCR) domains.

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Alle Produkte sind nur für Forschungszwecke bestimmt. Nicht für den menschlichen, tierärztlichen oder therapeutischen Gebrauch.

Menge: 0.1ml
Lieferbar: In stock
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