Mouse Anti-ADAMTS13 Recombinant Antibody (V2-179829) (CBMAB-A1192-YC)

Name: Mouse Anti-ADAMTS13 Recombinant Antibody (V2-179829)
SKU: CBMAB-A1192-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

V2-179829

Application

ELISA, WB

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.

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

More Infomation

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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

Isotype control

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

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