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AB28273

Anti-ADAMTS13 antibody

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Rabbit Polyclonal ADAMTS13 antibody. Suitable for WB, ICC/IF and reacts with Human samples. Immunogen corresponding to Synthetic Peptide within Human ADAMTS13.

View Alternative Names

C9orf8, UNQ6102/PRO20085, ADAMTS13, A disintegrin and metalloproteinase with thrombospondin motifs 13, ADAM-TS 13, ADAM-TS13, ADAMTS-13, von Willebrand factor-cleaving protease, vWF-CP, vWF-cleaving protease

2 Images
Immunocytochemistry/ Immunofluorescence - Anti-ADAMTS13 antibody (AB28273)
  • ICC/IF

Unknown

Immunocytochemistry/ Immunofluorescence - Anti-ADAMTS13 antibody (AB28273)

ICC/IF image of ab28273 stained HepG2 cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab28273, 1µg/ml) overnight at +4°C. The secondary antibody (green) was ab96899, DyLight® 488 goat anti-rabbit IgG (H+L) used at a 1/250 dilution for 1h.Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.

Western blot - Anti-ADAMTS13 antibody (AB28273)
  • WB

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Western blot - Anti-ADAMTS13 antibody (AB28273)

Glycosylation and the abundance of cysteine residues gives ADAMTS-13 an apparent molecular weight of about 190 kDa on reduced SDS PAGE gels.

All lanes:

Western blot - Anti-ADAMTS13 antibody (ab28273) at 1 µg/mL

Lane 1:

Recombinant Human ATS-13 at 0.08 µg

Lane 2:

Recombinant Human ATS-13 at 0.04 µg

Lane 3:

Recombinant Human ATS-13 at 0.02 µg

Predicted band size: 154 kDa

false

Key facts

Host species

Rabbit

Clonality

Polyclonal

Isotype

IgG

Carrier free

No

Reacts with

Human

Applications

ICC/IF, WB

applications

Immunogen

Synthetic Peptide within Human ADAMTS13. The exact immunogen used to generate this antibody is proprietary information.

Q76LX8

Specificity

ab28273 recognises the Carboxyterminal end of the long form of ADAMTS13.

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"}, "ICCIF" : {"fullname" : "Immunocytochemistry/ Immunofluorescence", "shortname":"ICC/IF"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/1000 - 1/5000", "WB-species-notes": "<p>1/1000, when using colorimetric substrates such as BCIP/NBT - 1/5000, when using chemiluminescent substrates. Detects a band of approximately 190 kDa. Glycosylation and the abundance of cysteine residues gives ADAMTS13 an apparent molecular weight of 190 kDa on reduced SDS PAGE gels. Several bands between 110-190 kDa are observed on Western blots, possibly indicating differentially processed ADAMTS 13. Dilution optimised using Chromogenic detection.</p>", "ICCIF-species-checked": "testedAndGuaranteed", "ICCIF-species-dilution-info": "1 µg/mL", "ICCIF-species-notes": "<p></p>" } } }
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Properties and storage information

Form
Liquid
Purification technique
Affinity purification Immunogen
Storage buffer
pH: 7.4 Constituents: PBS
Shipped at conditions
Blue Ice
Appropriate short-term storage duration
1-2 weeks
Appropriate short-term storage conditions
+4°C
Appropriate long-term storage conditions
-20°C
Aliquoting information
Upon delivery aliquot
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

ADAMTS13 also known as von Willebrand factor-cleaving protease (VWFCP) is a zinc-containing metalloprotease with a molecular mass of approximately 190 kDa. This protein belongs to the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs) family and exhibits a complex structure with specific domains including a metalloprotease and a disintegrin-like domain. ADAMTS13 is produced mainly in the liver and circulates in the blood plasma. Research shows that it primarily facilitates the cleavage of von Willebrand factor (vWF) a large multimeric protein essential for blood clotting.
Biological function summary

ADAMTS13 plays a role in regulating the size and function of von Willebrand factor (vWF) ensuring proper hemostatic balance. The enzyme prevents the accumulation of ultra-large vWF multimers which can lead to spontaneous platelet aggregation and thrombus formation. Although ADAMTS13 acts independently its function is closely linked to the dynamics of vWF in response to vascular injury. Properly functioning ADAMTS13 aids in maintaining normal blood flow by preventing unnecessary clot formation in the bloodstream.

Pathways

ADAMTS13 is critical in the coagulation and hemostatic pathways. It operates by modulating the activity of vWF which plays an essential role in platelet adhesion and aggregation forming part of the coagulation cascade. A significant relationship exists between ADAMTS13 and vWF in these pathways as the protease controls vWF multimer size directly impacting clot formation. In the context of hemostatic balance ADAMTS13 intersects with factors like thrombin and fibrinogen which further contribute to clotting processes.

ADAMTS13 deficiency or dysfunction is associated with thrombotic thrombocytopenic purpura (TTP) a rare but severe blood disorder. In TTP the reduced activity of ADAMTS13 leads to an over-accumulation of ultra-large vWF multimers resulting in excessive platelet aggregation and microvascular thrombosis. Another related condition is atypical hemolytic uremic syndrome (aHUS) where abnormal ADAMTS13 activity might exacerbate the disease pathology. Both disorders highlight the critical need for balanced ADAMTS13 function in maintaining vascular health and highlight its potential as a therapeutic target.
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Product protocols

For this product, it's our understanding that no specific protocols are required. You can visit:
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Target data

Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation.
See full target information ADAMTS13
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