Rabbit anti-Human ADAMTS13 Polyclonal Antibody | anti-ADAMTS13 antibody
ADAMTS13 Polyclonal Antibody
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Western Blot (WB)
(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.)
NCBI and Uniprot Product Information
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: 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