Target：ATS13

Gene Name：ADAMTS13 C9orf8 UNQ6102/PRO20085

Protein Name：A disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAM-TS 13) (ADAM-TS13) (ADAMTS-13) (EC 3.4.24.87) (von Willebrand factor-cleaving protease) (vWF-CP) (vWF-cleaving protease)

Human Gene Id：11093

Human Swiss Prot No：Q76LX8

Mouse Swiss Prot No：Q769J6

Immunogen：Synthesized peptide derived from human protein . at AA range: 940-1020

Specificity：ATS13 Polyclonal Antibody detects endogenous levels of protein.

Formulation：Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500-2000 ELISA 1:5000-20000

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Observed Band(KD)：156kD

Background： 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],

Function：catalytic activity:Cleaves the vWF at the 842-Tyr-|-Met-843 in the A2 domain of the vWF subunit.,cofactor:Binds 1 zinc ion per subunit.,cofactor:Binds 4 calcium ions .,disease:Defects in ADAMTS13 are the cause of congenital thrombotic thrombocytopenic purpura (TTP) [MIM:274150]; also known as Upshaw-Schulman syndrome (USS). Congenital TTP is a life-threatening systemic disorder due to constitutional deficiency of vWF-cleaving protease. Typical features are hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and nonfocal neurologic findings, decreased renal function and fever. Congenital TTP is characterized by neonatal onset, response to fresh plasma infusion and frequent relapses. Inheritance pattern is autosomal recessive. In sporadic cases, TTP is associated with deficiency of vWF-cleaving protease due to the presence of inhibiting autoantibodies (acquired T

Subcellular Location：Secreted . Secretion enhanced by O-fucosylation of TSP type-1 repeats.

Expression：Plasma. Expressed primarily in liver.