ADAMTS13, Polyclonal Antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Affinity Purification

Store at -20 degree C. Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Small volumes of anti-ADAMTS13 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Western Blot (WB)

WB 1:500 - 1:2000

Western blot analysis of extracts of various cell lines, using ADAMTS13 antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 5s.

154kDa

ADAM metallopeptidase with thrombospondin type 1 motif, 13

A disintegrin and metalloproteinase with thrombospondin motifs 13

A disintegrin and metalloproteinase with thrombospondin motifs 13

C9orf8; ADAM-TS 13; ADAM-TS13; ADAMTS-13; vWF-CP; vWF-cleaving protease  [Similar Products]

ATS13_HUMAN

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]

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

Chromosomal Location of Human Ortholog: 9q34

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

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

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

Disease: Thrombotic Thrombocytopenic Purpura, Congenital

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