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- Table of Contents
Facts about A disintegrin and metalloproteinase with thrombospondin motifs 13.
Human | |
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Gene Name: | ADAMTS13 |
Uniprot: | Q76LX8 |
Entrez: | 11093 |
Belongs to: |
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No superfamily |
A disintegrin and metalloproteinase with thrombospondin motifs 13; a disintegrin-like and metalloprotease (reprolysin type) with thrombospondintype 1 motif, 13; ADAM metallopeptidase with thrombospondin type 1 motif, 13; ADAMTS13; ADAM-TS13; ADAMTS-13; DKFZp434C2322; EC 3.4.24.14; EC 3.4.24.82; EC 3.4.24.87; FLJ42993; MGC118899; MGC118900; TTP; TTPADAM-TS 13; vWF-cleaving protease; vWF-CP; vWF-CPC9orf8; VWFCPvon Willebrand factor-cleaving protease
Mass (kDA):
153.604 kDA
Human | |
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Location: | 9q34.2 |
Sequence: | 9; NC_000009.12 (133414339..133459403) |
Plasma. Expressed primarily in liver.
Secreted. Secretion enhanced by O-fucosylation of TSP type-1 repeats.
Boster Bio's anti-ADAMTS13 antibody (catalog number A00586) reacts with Human in ELISA and flow cytometry. Boster Bio validates all of its antibodies against known positive and negative samples. It is a reliable predictor of the likelihood of relapse among people suffering from ADAMTS13 deficiency.
ADAMTS13 is an thrombospondin-type 1 motif disintegrin and metalloproteinase. Although it is a key component of coagulation markers however, it is not available immediately during initial screening. However it is a crucial differentiation factor among the various disease conditions that may be present in TTP patients. Inflammation and illness of severe severity can adversely affect the survival of ADAMTS13 and production.
This marker of coagulation is used as a part the hemostasis pathway. ADAMTS13's capability to recognize and break down large VWF multimers inhibits the activities of thrombin (a protein that functions as a prothrombotic molecule in the blood) and plasmin (two proteins that inhibit the function of thrombin). This suggests the possibility of ADAMTS13 playing a role in the vascular site of injuries.
In a recent study researchers examined thrombotic microangiopathy (TMA) in patients with severe and malignant hypertension. They discovered that ADAMTS13 activity in malignant hypertension patients was lower than in control. In addition there was an unfavorable correlation between the levels of active VWF and platelet count in hypertension patients. The authors concluded that ADAMTS13 activity may be a key component in malignant hypertension.
Incredibly, ADAMTS13 activity was decreased in various medical conditions, including neonatal age and liver cirrhosis. However, the amount of this coagulation marker is typically low. This indicates that patients with ADAMTS13 deficiencies are not at higher risk of clot formation in the event of anticoagulation being used. The study results are promising and warrant further investigation.
ADAMTS13 activity has been found to be significantly correlated with serum aminotransferases, liver stiffness, and aspartate aminotransferase-to-platelet ratio. These findings suggest that ADAMTS13's activity could be a good indicator to identify the development of HCC in chronic hepatitis B sufferers. These findings are likely to be confirmed in future studies. This blood test could play an important part in diagnosis and treatment.
COVID-19 can also increase the risk of thrombotic episodes. The mechanisms that lead to the development of a prothrombotic condition are not fully understood. A study on COVID-19 patients analyzed the plasma ADAMTS13 activity and von Willebrand factor antigen levels in order to establish a prognostic marker. The results showed that both of these markers have an important role to play in the thrombosis of the vascular system.
Moreover, both VWF13 and ADAMTS13 were associated with an increased risk of developing heart disease in older adults. The relative predictive value of these markers is not yet established. Further research is needed to determine the mechanisms that underlie this connection. There are several promising studies that have been done regarding the role of ADAMTS13 and VWF in preventing heart events. As a screening tool it is possible to identify a number of prognostic factors.
ADAMTS13, a gene that is expressed on stellate cells of the liver is associated with severe sepsis aswell as non-infectious systemic inflammatory response syndrome. Patients with ADAMTS13 deficiency have a reduced severity and a better outcome. A severe ADAMTS13 deficiency is associated with an increased risk of relapse. A severe ADAMTS13 deficits can cause hemolytic anemia, which requires appropriate treatment and follow-up in order to prevent serious complications.
A meta-analysis of five studies and 1500 individual cases found a link between low ADAMTS13 activity, increased risk of ischemic stroke and CHD. Also, patients with low ADAMTS13 activity were at a higher risk of developing stroke ischemic compared to those with higher levels of the protein. The study also demonstrated an unidirectional correlation between low levels ADAMTS13 activity and an increased risk of coronary disease.
ADAMTS13 laboratory testing provides important information for TTP diagnosis. ADAMTS13 activity is the principalstay of TTP testing. Antibodies to the gene have been created and are supported by an increasing body of medical literature. ADAMTS13 activity test performance and its clinical application are important for laboratory professionals. Lab professionals can greatly assist in the treatment of TTP patients by providing useful clinical information to doctors.
Comparison of ADAMTS13 activity between patients suffering from chronic HD and those without chronic HD has found a significant correlation between chronic HD events and cardiovascular events. The activity of ADAMTS13 HD has been linked to numerous cardiovascular risk factors like hypertension and diabetes. It has also been linked with newly developed coronary heart disease.
ADAMTS13 activity was significantly reduced in the early stages of trauma, a condition associated with coagulopathy, DIC and the necessity for FFP transfusion. Despite this , ADAMTS13 activity did not decrease in severe trauma patients. AT activity is positively related to it. Therefore, it is believed to be an indicator for severe ADAMTS13 deficiency in the near future.
In mice, the disease is often inactive and can not be seen until the age of. Rare ADAMTS13 deficiency patients generally do not have symptoms until the third decade of life. Genetically deficient people may experience the loss of ADAMTS13 activity but not an complete ADAMTS13 activity defect.
Relapse risk can be predicted by measuring ADAMTS13 IgG levels in plasma that are measured at predefined intervals. In a retrospective study only two-thirds patients with TTP had the stage of remission. Plasma was taken from these patients at different intervals, with the median being 10 months, and the range ranging from one to 96 months. Patients were then monitored for up to 22 consecutive months , until the relapse. The median time was 21 months. This relapse occurred 21 months after the first episode. A prospective multicenter cohort study that includes repeated blood samples could determine if the ADAMTS13 marker is a useful predictor of relapse.
It has been suggested that ADAMTS13 could be a new predictor of relapse risk for TTP patients suffering from the disease. This study offers evidence of a changing immune response to ADAMTS13 in TTP patients. Although the ADAMTS13 marker could be a novel indicator for the risk of relapse, it is being studied.
Prospective studies are required prior to the ADAMTS13 marker is used to determine the risk of relapse for patients suffering from lung cancer. A small study on mice showed that the recombinant human ADAMTS13 reduced the size of cerebral infarction. These findings support the notion of ADAMTS13 as a reliable indicator of the likelihood of relapse for patients suffering from lung cancer.
However, the research is not able to predict relapse risk in idiopathic TTP. The majority of patients experience an exacerbation within 30 days after their last plasma exchange. This is despite the fact that it's impossible to know which patients are at higher risk. The study included acute episodes of 44 in 26 patients. This result is constant and can be replicated.
ADAMTS13 activity levels in plasma are controlled by multiple physiological conditions. The study was carried out in accordance with the Declaration of Helsinki and informed consent was obtained from all patients. The assay for ADAMTS13 activity is a complex procedure that requires the expertise of personnel in the laboratory. ADAMTS13 activity levels in plasma are influenced by bilirubin and free hemoglobin Certain denaturants could be able to falsely increase ADAMTS13 activity.
Rhythm stability and the VWF/ADAMTS13 ratio are also affected by an increase in ADAMTS13 levels. However, the presence of RVO or PTS is not a guarantee of that there is a relapse. The index needs to be confirmed before it can be widely used to predict the possibility of VTE returning.
PMID: 11574066 by Soejima K., et al. A novel human metalloprotease synthesized in the liver and secreted into the blood: possibly, the von Willebrand factor-cleaving protease?
PMID: 11557746 by Zheng X., et al. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura.