Mouse anti-Human ADAMTS-13 Monoclonal Antibody | anti-ADAMTS13 antibody

ADAMTS-13 (Von Willebrand Factor-Cleaving Protease (VWFCP))

Cat. Num. AAA631743

• Gene Names: ADAMTS13; TTP; VWFCP; C9orf8; vWF-CP; FLJ42993; MGC118899; MGC118900; DKFZp434C2322
• Reactivity: Human
• Applications: ELISA, Western Blot, Immunofluorescence
• Purity: Affinity Purified; Purified by Protein G affinity chromatography.
• Synonyms: ADAMTS-13; Monoclonal Antibody; ADAMTS-13 (Von Willebrand Factor-Cleaving Protease (VWFCP)); Anti -ADAMTS-13 (Von Willebrand Factor-Cleaving Protease (VWFCP)); anti-ADAMTS13 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG1
• Clone Number: 20A5
• Specificity: Recognizes ADAMTS-13. Reacts with the central to C-terminal TSP-1 repeats 2-5 of ADAMTS-13 (aa686-894). Species Crossreactivity: Human
• Purity/Purification: Affinity Purified; Purified by Protein G affinity chromatography.
• Form/Format: Supplied as as liquid in PBS, 0.1% BSA, 0.02% sodium azide.

• Applicable Applications for anti-ADAMTS13 antibody: ELISA (EL/EIA), Western Blot (WB), Immunofluorescence (IF)
• Application Notes: Suitable for use in Immunofluorescence, ELISA and Western Blot.; Dilution: ELISA: 1:50; Western Blot: 1:50 under non-reducing conditions.; Immunofluorescence: 1:50
• Immunogen: Recombinant corresponding to full length ADAMTS-13.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Related Product Information for anti-ADAMTS13 antibody

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 190kD 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 140kD, 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.

Product Categories/Family for anti-ADAMTS13 antibody

Antibodies; Abs to Matrix Metalloproteinases

NCBI and Uniprot Product Information

• NCBI GI #: 73695936
• NCBI GeneID: 11093
• NCBI Accession #: NP_620596.2
• NCBI GenBank Nucleotide #: NM_139027.3
• UniProt Accession #: Q6QNA4; Q6QNA5; Q6QNA6; Q6QNA7; Q76LX8; Q9UGQ1
• Molecular Weight: 6,844 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: TTP; VWFCP; C9orf8; vWF-CP; FLJ42993; MGC118899; MGC118900; DKFZp434C2322
• NCBI Protein Information: A disintegrin and metalloproteinase with thrombospondin motifs 13; ADAM-TS13; ADAMTS-13; ADAM-TS 13; OTTHUMP00000022496; OTTHUMP00000022497; OTTHUMP00000022498; vWF-cleaving protease; von Willebrand factor-cleaving protease; a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13
• UniProt Protein Name: Von Willebrand factor-cleaving protease
• Protein Family: A disintegrin and metalloproteinase with thrombospondin motifs
• UniProt Gene Name: ADAMTS13
• UniProt Entry Name: Q6QNA4_HUMAN

NCBI Description

This gene encodes a member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motif) protein family. Members of the family share several distinct protein modules, including a propeptide region, a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 (TS) motif. Individual members of this family differ in the number of C-terminal TS motifs, and some have unique C-terminal domains. The enzyme encoded by this gene is the von Willebrand Factor (vWF)-cleaving protease, which is responsible for cleaving at the site of Tyr842-Met843 of the vWF molecule. A deficiency of this enzyme is associated with thrombotic thrombocytopenic purpura. Alternative splicing of this gene generates multiple transcript variants encoding different isoforms. [provided by RefSeq]

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

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; peptide catabolic process; response to toxin; cell-matrix adhesion; protein processing; proteolysis; post-translational protein modification

Disease: Thrombotic Thrombocytopenic Purpura, Congenital