ADAMTS13 Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	ELISA, IHC, WB
Recommend Dilution	ELISA.  Western Blot: 1/500-1/2000. Immunohistochemistry: 1/50-1/500.
Reactivity	Human, Mouse
Host	Rabbit
Clonality	Polyclonal
Immunogen	Synthetic peptide derived from N-terminal domain of ADAM-TS13 protein
Specificity	Reacts specifically with Human 153 kDa ADAMTS13 protein. Cross reacts with Mouse protein.
Formulation	State: Serum State: Lyophilized Serum Preservative: None
Reconstitution Method	Restore in distilled water to initial volume.
Conjugation	Unconjugated
Storage Condition	Store lyophilized at 2-8°C for 6 months or at -20°C long term. After reconstitution store the antibody undiluted at 2-8°C for one month  or (in aliquots) at -20°C long term. Avoid repeated freezing and thawing.
Predicted Protein Size	154 kDa
Gene Name	ADAM metallopeptidase with thrombospondin type 1 motif 13
Database Link	Entrez Gene 11093 Human UniProt ID Q76LX8
Background	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 µg/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.
Synonyms	ADAMTS-13, ADAM-TS13, C9orf8, vWF-cleaving protease
Reference Data

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