ADAMTS13 Antibody

Code CSB-PA001301LA01HU
Size US$299Purchase it in Cusabio online store
(only available for customers from the US)
Image
  • IHC image of CSB-PA001301LA01HU diluted at 1:400 and staining in paraffin-embedded human liver tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

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Product Details

Full Product Name Rabbit anti-Homo sapiens (Human) ADAMTS13 Polyclonal antibody
Uniprot No. Q76LX8
Target Names ADAMTS13
Alternative Names A disintegrin and metalloproteinase with thrombospondin motifs 13 antibody; A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif 13 antibody; A disintegrin like and metalloprotease with thrombospondin type 1 motif 13 antibody; ADAM metallopeptidase with thrombospondin type 1 motif 13 antibody; ADAM TS antibody; ADAM-TS 13 antibody; ADAM-TS13 antibody; ADAMTS 13 antibody; ADAMTS-13 antibody; ADAMTS13 antibody; ADAMTS13 protein antibody; ATS13_HUMAN antibody; C9orf8 antibody; TTP antibody; Von Willebrand factor cleaving protease antibody; von Willebrand factor-cleaving protease antibody; vWF cleaving protease antibody; vWF CP antibody; vWF-cleaving protease antibody; vWF-CP antibody; vWFCP antibody
Raised in Rabbit
Species Reactivity Human
Immunogen Recombinant Human A disintegrin and metalloproteinase with thrombospondin motifs 13 protein (738-905AA)
Immunogen Species Homo sapiens (Human)
Conjugate Non-conjugated
Clonality Polyclonal
Isotype IgG
Purification Method >95%, Protein G purified
Concentration It differs from different batches. Please contact us to confirm it.
Buffer Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form Liquid
Tested Applications ELISA, IHC
Recommended Dilution
Application Recommended Dilution
IHC 1:200-1:500
Protocols ELISA Protocol
Immunohistochemistry (IHC) Protocol
Troubleshooting and FAQs Antibody FAQs
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
(From Uniprot)
Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation.
Gene References into Functions
  1. acute myeloid leukemia patients with low activity of ADAMTS-13 had worse prognosis after bone morrow transplantation. PMID: 30322352
  2. The Plasma Levels of ADAMTS-13, von Willebrand Factor, VWFpp, and Fibrin-Related Markers in Patients With Systemic Sclerosis Having Thrombosis PMID: 29130325
  3. The aim of the study was to investigate the role of von Willebrand factor (vWF), the vWF-cleaving protease, ADAMTS13, the composition of thrombus, and patient outcome following mechanical cerebral artery thrombectomy in patients with acute ischemic stroke. PMID: 29887594
  4. ADAMTS 1, 4, 12, and 13 levels in the maternal and cord blood were lower in the preeclampsia group than in the control group. ADAMTS 1, 4, and 12 levels in placental tissues were higher in the preeclampsia group. PMID: 29135310
  5. Decreased ADAMTS-13 activity was found in patients with proliferative lupus nephritis, and plasma ADAMTS-13 activity was closely associated with renal injury indices. PMID: 28786769
  6. These results suggest that highly elevated plasma VWF might accelerate platelet thrombus formation not only in the circulation but also on the surface of vascular endothelial cells in the setting of ADAMTS13 deficiency in Upshaw-Schulman syndrome. PMID: 29040872
  7. Prothrombotic state and systemic inflammation status might contribute to explaining the high incidence of concealed chronic renal failure in COPD, and plasma ADAMTS-13 levels may serve as a strong predictor. PMID: 29255356
  8. VWF, GMP-140, ADAMTS13 and the cerebral vasospasm, delayed cerebral ischemia, tumor diameter and prognosis of aneurysmal subarachnoid hemorrhage patients are closely related PMID: 29077161
  9. The levels of ADAMTS13 among neonates were higher as compared with healthy adults, despite a significant elevation of VWF antigen (Ag) and Ristocetin cofactor (RiCof) noted in all neonates. PMID: 28087247
  10. investigated the roles of ADAMTS13 and VWF in thrombotic events of patients with Connective Tissue Diseases PMID: 26759371
  11. Studied the significance of the von Willebrand factor (VWF)/ ADAMTS-13 ratio in advanced non-small-cell lung cancer (NSCLC). Findings suggest that the imbalance between VWF secretion and ADAMTS-13 may play a role in the hypercoagulability state in advanced NSCLC, and increase of the plasma VWF/ADAMTS-13 ratio may serve as an independent predictive factor for mortality in patients with advanced NSCLC. PMID: 28374895
  12. vaso-occlusive crisis in sickle cell disease is associated with increased reactivity of VWF, without a pronounced ADAMTS-13 deficiency PMID: 28457019
  13. The endogenous plasmin activation alone is not sufficient to cause Thrombotic thrombocytopenic purpura (TTP), but plasmin activation with ADAMTS13 deficiency might increase the risk of TTP onset. PMID: 29228282
  14. the N-linked glycans of ADAMTS-13 play a crucial role in regulating ADAMTS-13 activity PMID: 28370891
  15. Complex VWF-ADAMTS13-mediated mechanisms disturb haemostasis in inflammatory bowel disease. PMID: 28765701
  16. Missense variant in ADAMTS13 gene in a patient with NCIPH decreases secretion and activity of ADAMTS13 protein. PMID: 28980147
  17. Deficiency of the von Willebrand factor-cleaving protease ADAMTS13 is central to the pathophysiology of thrombotic thrombocytopenic purpura. Reviewed is the evidence emerged from epidemiological studies of an inverse relationship between the plasma levels of ADAMTS13 and the risk of acute coronary syndrome. [review] PMID: 28521259
  18. Data indicate that in cultured endothelial cells, one role of endogenous ADAMTS-13 is regulation of angiogenesis, mediated through VEGF and AKT signaling pathway. PMID: 28546076
  19. ADAMTS13 haplotype had an independent protective effect on CAD and genetic variation of vWF V1565L polymorphism modulates ADAMTS13 activity. PMID: 27536857
  20. The relative deficiency of plasma ADAMTS13 activity in subarachnoid haemorrhage patients may associate with worse outcome. PMID: 28102428
  21. Low ADAMTS13 was associated with increased mortality in patients with severe sepsis and septic shock and was comparable to APACHE II scores for predicting mortality. PMID: 28447577
  22. These observations support the hypothesis that a significantly reduced ADAMTS13/VWF ratio in the coronary artery flow plays a pathogenic role in acute coronary syndromes (ACS) and suggest that transition from laminar to turbulent flow at sites of coronary stenosis further enhances VWF activation and deposition. PMID: 27034431
  23. Low ADAMTS-13 activity is associated with an increased risk of coronary heart disease in the elderly, independently of VWF and established cardiovascular risk factors PMID: 27559008
  24. As folding stability was progressively disrupted, proteolysis by ADAMTS13 increased. Due to the range of folding stabilities and wide distribution of VWF A2 domain mutations studied, we conclude that these mutations disrupt regulated folding of the VWF A2 domain PMID: 29186156
  25. The 'closed' conformation of ADAMTS-13 restricts its specificity and protects against fibrinogenolysis PMID: 27514025
  26. Our results indicate that the secondary TMA syndrome and its poor outcome is characterized by relative ADAMTS13 deficiency, inflammation, and complement activation with consumption via the classical and alternative pathways. PMID: 27771173
  27. Diagnosis of thrombotic thrombocytopenic purpura among patients with ADAMTS13 Activity 10%-20. PMID: 28815685
  28. Rare genetic variants in the ADAMTS13 on Willebrand factor-binding domain contribute to pediatric stroke. PMID: 27412500
  29. No correlation was found between VWF/ADAMTS13 and infarct size in patients. Patients suffering from intramyocardial hemorrhage had significantly higher VWF activity and lower ADAMTS13 activity. Intracoronary administration of rADAMTS13 did not decrease infarct size or IMH in a porcine model of myocardial ischaemia-reperfusion, disputing the idea that the imbalance in ADAMTS13 and VWF as the cause of no reflow. PMID: 27174213
  30. both in acute and chronic cerebrovascular disease patients, ADAMTS13 levels were significantly decreased, with the lowest ADAMTS13 levels found in acute stroke patients. This difference was even more distinct when the ratio of VWF:ADAMTS13 was considered. These results demonstrate the potentially important involvement of the VWF/ADAMTS13 axis in ischemic stroke. PMID: 28591212
  31. Placental trophoblasts and villous vessel endothelial cells produce a full-length and functional ADAMTS13 protease. Placental expression of ADAMTS13 exhibits a dynamic change during pregnancy, which seems to be inhibited in late pregnancy and in patients with severe preeclampsia. PMID: 28751574
  32. ADAMTS13 activity appears to be an independent risk factor for incident prediabetes and type 2 diabetes. PMID: 27787621
  33. both anti-ADAMTS13 IgG antibody and ADAMTS13 antigen levels correlate with outcome in thrombotic thrombocytopenic purpura with increased cardiac and neurological involvement and increased mortality. PMID: 28576877
  34. Low ADAMTS-13 levels correlated with high levels of NTproBNP but had no independent prognostic significance. In conclusion, high VWF:Ag levels, probably representing endothelial dysfunction, are associated with prognosis in patients with AL amyloidosis, independently of other features of the disease or cardiac biomarkers. PMID: 27166361
  35. findings highlight the complexity of glycan modifications on ADAMTS13, which may have implications for its interaction with immune- or clearance receptors containing carbohydrate recognition domains. PMID: 27574189
  36. ADAMTS-13 levels are decreased in plasma of AML patients and the level of ADAMTS-13 is related to inflammation and infection of AML patients. Besides, low ADAMTS-13 level is one potential risk factor for AML PMID: 28033504
  37. these results demonstrate that HNPs1-3 may be potent inhibitors of ADAMTS13 activity, likely by binding to the central A2 domain of VWF and physically blocking ADAMTS13 binding. PMID: 27207796
  38. Three Single Nucleotide Variants (p.Val154Ile, p.Asp187His and p.Arg421Cys) showed reduced ex vivo and in vitro ADAMTS13 levels. However, the low frequency of these variants makes it difficult to confirm their association with Deep Vein Thrombosis. PMID: 27802307
  39. ADAMTS13 activity and VWF:Ag levels are both associated with an increased risk of all-cause and cardiovascular mortality. PMID: 27737864
  40. These findings support an ADAMTS13 activation model in which VWF D4-CK engages the TSP8-CUB2 domains, inducing the conformational change that disrupts the CUB1-spacer domain interaction and thereby activates ADAMTS13 PMID: 28209710
  41. Three cases of systemic lupus erythematosus with ADAMTS13 inhibitor-negative thrombotic microangiopathy were successfully treated with combination of mycophenolate mofetil, plasma exchange and steroid. [case reports] PMID: 27416846
  42. The pregnancy loss rate does not appear to be affected by both ADAMTS-13 and ADAMTS-19. PMID: 28088271
  43. Two novel ADAMTS13 mutations (p.I143T and p.Y570C) identified in two adolescence onset congenital thrombotic thrombocytopenic purpura patients were studied. Proteasome degradation of these ADAMTS13 mutants contributed to their reduced secretion. PMID: 27665541
  44. Correlation between ADAMTS13 activity and neurological impairment in acute thrombotic microangiopathy patients. PMID: 27379499
  45. review to introduce the state of progress with respect to some of the theorized roles of ADAMTS13[review] PMID: 27696191
  46. IL-8, TNF-alpha, tissue factor, IL-8+tissue factor and TNF-alpha+tissue factor decreased the levels of ADAMTS13 secreted by umbilical vein endothelial cells. PMID: 27766025
  47. Significantly lower ADAMTS-13 levels and significantly higher VWF antigen levels were concluded to be the result of a pathological process rather than an etiological factor for Venous thromboembolism. PMID: 26872106
  48. ADAMTS13 and VWF are co-expressed in microvascular endothelial cells. PMID: 26366828
  49. ADAMTS13-a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13-regulates a key physiological process of coagulation in the circulation by cleaving VWF multimers into small, inactive fragments. Low levels of ADAMTS13 in the blood may play a role in cardiovascular and hematological disorders, and clarifying its role may help improve disease management. PMID: 27746209
  50. ADAMTS13 activity, d-Dimer and cystatin C are associated with retinopathy in type 1 diabetic patients and are promising biomarkers for the diagnosis and monitoring of diabetic retinopathy PMID: 27208743

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Involvement in disease Thrombotic thrombocytopenic purpura congenital (TTP)
Subcellular Location Secreted
Tissue Specificity Plasma. Expressed primarily in liver.
Database Links

HGNC: 1366

OMIM: 274150

KEGG: hsa:11093

STRING: 9606.ENSP00000360997

UniGene: Hs.131433

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