Mouse anti-Human ADAMTS-13 Monoclonal Antibody | anti-ADAMTS-13 antibody

ADAMTS-13, Human, mAb 20A5

Cat. Num. AAA584031

• Gene Names: ADAMTS13; VWFCP; C9orf8; vWF-CP; ADAM-TS13; ADAMTS-13
• Reactivity: Human
• Applications: Immunofluorescence, Western Blot
• Synonyms: ADAMTS-13; Monoclonal Antibody; Human; mAb 20A5; Von Willebrand Factor-Cleaving Protease (VWFCP); anti-ADAMTS-13 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: Mouse IgG1
• Form/Format: 1 ml (100 ug/ml) 0.2 um filtered antibody solution in PBS, containing 0.1% bovine serum albumin and 0.02% sodium azide.; For unit size 0.5 mg or larger: PBS only (concentration< 0.5mg/ml)
• Sequence Length: 1371

• Applicable Applications for anti-ADAMTS-13 antibody: Immunoassay (IA), Immunofluorescence (IF), Western Blot (W)
• Application Notes: Application Use: For Western blotting and immunofluorescence dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions. The typical starting working dilution is 1:50.
• Preparation and Storage: Product should be stored at 4 degree C. Under recommended storage conditions, product is stable for one year.

Related Product Information for anti-ADAMTS-13 antibody

Product Description: The monoclonal antibody 20A5 recognizes human ADAMTS-13, A Disintegrin And Metalloprotease with ThromboSpondin type 1 domain 13. ADAMTS-13 is produced by hepatic stellate cells and in smaller amounts by human endothelial cells, and is present in plasma at a concentration of approximately 1 ug/ml. ADAMTS-13 is a zinc-containing metalloprotease belonging to the ADAMTS family characterized by a protease domain, an adjacent disintegrin-like domain, one or more thrombospondin type 1 repeats, a cystein-rich domain and a typical spacer region. ADAMTS-13 is composed of a series of domains (amino to carboxy terminal): metalloprotease, disintegrin-like, central thrombospondin-1 (TSP-1), cysteine-rich, spacer, seven additional TSP-1 domains and two unique CUB domains. ADAMTS-13 has no hydrophobic transmembrane domain, and hence it is not anchored in the cell membrane. The apparent molecular weight is 170 or 190 kDa on non-reducing or reducing SDS-PAGE, respectively. ADAMTS-13 has an important function in haemostasis, where it catalyzes the cleavage of the peptide bond between tyrosine-1605 and methionine-1606 in the A2 domain of von Willebrand Factor (VWF), resulting in 2 electrophoretic reduced fragments of 176 and 140 kDa, respectively. This process renders large multimers less adhesive and hence less reactive in the setting of thrombus formation. ADAMTS-13 is therefore said to be a natural anti-thrombotic agent. Severe ADAMTS-13 deficiency is associated with systemic microvascular thrombosis in familial or acquired thrombotic thrombocytopenic purpura (TTP). The accumulation of non-cleaved large VWF multimers causes spontaneous systemic platelet aggregation blocking oxygen supply to vital organs. This life-threatening disorder can lead to ischemic disease with (multiple) organ failure. The monoclonal antibody 20A5 recognizes the central to C-terminal TSP-1 repeats 2 to 5 of ADAMTS-13 (amino acid 686-894).

NCBI and Uniprot Product Information

• NCBI GI #: 73695936
• NCBI GeneID: 11093
• NCBI Accession #: NP_620596.2
• NCBI GenBank Nucleotide #: NM_139027.4
• Molecular Weight: 39,864 Da
• NCBI Official Full Name: A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform 2 preproprotein
• NCBI Official Synonym Full Names: ADAM metallopeptidase with thrombospondin type 1 motif, 13
• NCBI Official Symbol: ADAMTS13
• NCBI Official Synonym Symbols: VWFCP; C9orf8; vWF-CP; ADAM-TS13; ADAMTS-13
• NCBI Protein Information: A disintegrin and metalloproteinase with thrombospondin motifs 13; vWF-cleaving protease; von Willebrand factor-cleaving protease; a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13
• UniProt Protein Name: A disintegrin and metalloproteinase with thrombospondin motifs 13
• UniProt Gene Name: ADAMTS13
• UniProt Synonym Gene Names: C9orf8; UNQ6102/PRO20085; ADAM-TS 13; ADAM-TS13; ADAMTS-13; vWF-CP; vWF-cleaving protease
• UniProt Entry Name: ATS13_HUMAN

NCBI Description

This gene encodes 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 Factor (vWF). Defects in this gene are associated with thrombotic thrombocytopenic purpura. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2013]

Uniprot Description

ADAMTS13: Cleaves the vWF multimers in plasma into smaller forms. Defects in ADAMTS13 are the cause of thrombotic thrombocytopenic purpura congenital (TTP); also known as Upshaw-Schulman syndrome (USS). A hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Motility/polarity/chemotaxis; Extracellular matrix; Secreted, signal peptide; Secreted; EC 3.4.24.87; Calcium-binding; Protease

Chromosomal Location of Human Ortholog: 9q34

Cellular Component: extracellular space; proteinaceous extracellular matrix; cell surface; endoplasmic reticulum lumen

Molecular Function: integrin binding; protein binding; metallopeptidase activity; zinc ion binding; metalloendopeptidase activity; calcium ion binding

Biological Process: integrin-mediated signaling pathway; protein amino acid O-linked glycosylation; platelet activation; cellular protein metabolic process; glycoprotein metabolic process; cell-matrix adhesion; peptide catabolic process; response to toxin; protein processing; proteolysis; post-translational protein modification

Disease: Thrombotic Thrombocytopenic Purpura, Congenital