Recombinant Human A disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13), partial

Code CSB-YP748481HU
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Source Yeast
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Code CSB-EP748481HU
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Source E.coli
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Code CSB-EP748481HU-B
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Source E.coli
Conjugate Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
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Code CSB-BP748481HU
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Source Baculovirus
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Code CSB-MP748481HU
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Source Mammalian cell
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Product Details

Purity
>85% (SDS-PAGE)
Target Names
ADAMTS13
Uniprot No.
Alternative Names
A disintegrin and metalloproteinase with thrombospondin motifs 13; A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif 13; A disintegrin like and metalloprotease with thrombospondin type 1 motif 13; ADAM metallopeptidase with thrombospondin type 1 motif 13; ADAM TS; ADAM-TS 13; ADAM-TS13; ADAMTS 13; ADAMTS-13; ADAMTS13; ADAMTS13 protein; ATS13_HUMAN; C9orf8; TTP; Von Willebrand factor cleaving protease; von Willebrand factor-cleaving protease; vWF cleaving protease; vWF CP; vWF-cleaving protease; vWF-CP; vWFCP
Species
Homo sapiens (Human)
Protein Length
Partial
Tag Info
Tag type will be determined during the manufacturing process.
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Form
Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer before Lyophilization
Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Reconstitution
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting and FAQs
Storage Condition
Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time
Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet
Please contact us to get it.

Customer Reviews and Q&A

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Target Background

Function
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. Note=Secretion enhanced by O-fucosylation of TSP type-1 repeats.
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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