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Mouse Anti-ADAMTS13 Recombinant Antibody (V2-179827) (CBMAB-A1190-YC)

Provided herein is a Mouse monoclonal antibody against Human ADAM Metallopeptidase With Thrombospondin Type 1 Motif 13. The antibody can be used for immunoassay techniques, such as ELISA, IF, WB.
See all ADAMTS13 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
V2-179827
Antibody Isotype
IgG1
Application
ELISA, IF, WB

Basic Information

Immunogen
C-terminal TSP-1 repeats 2 to 5 of ADAMTS-13 (amino acid 686-894).
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:50
IF(ICC)1:50

Formulations & Storage [For reference only, actual COA shall prevail!]

Buffer
PBS, 0.1% BSA
Preservative
0.02% sodium azide
Concentration
0.1 mg/ml
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
aa 686-894

Target

Full Name
ADAM Metallopeptidase With Thrombospondin Type 1 Motif 13
Introduction
ADAMTS13 is a member of a family of proteins containing several distinct regions, including a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 (TS) motif. The enzyme encoded by this gene specifically cleaves von Willebrand
Entrez Gene ID
11093
UniProt ID
Q76LX8
Alternative Names
ADAM Metallopeptidase With Thrombospondin Type 1 Motif 13; A Disintegrin-Like And Metalloprotease (Reprolysin Type) With Thrombospondin Type 1 Motif, 13; Von Willebrand Factor-Cleaving Protease; VWF-Cleaving Protease; ADAM-TS13; ADAMTS-13; C9orf8; VWF-CP;
Function
Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation.
Biological Process
Cell-matrix adhesion
Cellular response to interferon-gamma
Cellular response to interleukin-4
Cellular response to lipopolysaccharide
Cellular response to tumor necrosis factor
Extracellular matrix organization
Glycoprotein metabolic process
Integrin-mediated signaling pathway
Peptide catabolic process
Platelet activation
Protein processing
Proteolysis
Response to amine
Response to potassium ion
Response to toxic substance
Cellular Location
Secreted. Secretion enhanced by O-fucosylation of TSP type-1 repeats.
Involvement in disease
Thrombotic thrombocytopenic purpura, hereditary (TTP): An autosomal recessive hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever.
PTM
Glycosylated. O-fucosylated by POFUT2 on a serine or a threonine residue found within the consensus sequence C1-X2-(S/T)-C2-G of the TSP type-1 repeat domains where C1 and C2 are the first and second cysteine residue of the repeat, respectively. Fucosylated repeats can then be further glycosylated by the addition of a beta-1,3-glucose residue by the glucosyltransferase, B3GALTL. Fucosylation mediates the efficient secretion of ADAMTS13. May also be C-glycosylated on tryptophan residues within the consensus sequence W-X-X-W of the TPRs, and also N-glycosylated. These other glycosylations can also facilitate secretion.
The precursor is processed by a furin endopeptidase which cleaves off the pro-domain.

Bortot, M., Ashworth, K., Sharifi, A., Walker, F., Crawford, N. C., Neeves, K. B., ... & Di Paola, J. (2019). Turbulent flow promotes cleavage of vWF (von Willebrand factor) by ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type-1 motif, member 13). Arteriosclerosis, thrombosis, and vascular biology, 39(9), 1831-1842.

Takaya, H., Namisaki, T., Kitade, M., Kaji, K., Nakanishi, K., Tsuji, Y., ... & Yoshiji, H. (2019). VWF/ADAMTS13 ratio as a potential biomarker for early detection of hepatocellular carcinoma. BMC gastroenterology, 19(1), 1-10.

Alias, H., Yong, W. L., Muttlib, F. A. A., Koo, H. W., Loh, C. K., Lau, S. C. D., ... & Azma, R. Z. (2018). Acquired thrombotic thrombocytopenia purpura associated with severe ADAMTS13 deficiency in a 3-year-old boy: a case report and review of the literature. Journal of medical case reports, 12(1), 1-8.

Ercig, B., Wichapong, K., Reutelingsperger, C. P., Vanhoorelbeke, K., Voorberg, J., & Nicolaes, G. A. (2018). Insights into 3D structure of ADAMTS13: a stepping stone towards novel therapeutic treatment of thrombotic thrombocytopenic purpura. Thrombosis and haemostasis, 118(01), 028-041.

Takaya, H., Yoshiji, H., Kawaratani, H., Sakai, K., Matsumoto, M., Fujimura, Y., & Fukui, H. (2017). Decreased activity of plasma ADAMTS13 are related to enhanced cytokinemia and endotoxemia in patients with acute liver failure. Biomedical reports, 7(3), 277-285.

Robinson, C. R., Pieper, I. L., & Kanamarlapudi, V. (2017). A disintegrin and metalloproteinase with thrombospondin motifs-13; ADAMTS13; ADAMTS-13; von Willebrand factor-cleaving protease; vWF-cp.

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For research use only. Not intended for any clinical use.

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