protein

ADAMTS13, Recombinant, Human (A disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13)

MBS636953

0.02 mg

740 USD

Recombinant Protein

ADAMTS13, Recombinant, Human (A disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13)

[ADAMTS13]

[A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform 2 preproprotein; A disintegrin and metalloproteinase with thrombospondin motifs 13; A disintegrin and metalloproteinase with thrombospondin motifs 13; vWF-cleaving protease; von Willebrand factor-cleaving protease; a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13; ADAM metallopeptidase with thrombospondin type 1 motif, 13; von Willebrand factor-cleaving protease]

[ADAMTS13; ADAMTS13; VWFCP; C9orf8; vWF-CP; ADAM-TS13; ADAMTS-13; C9orf8; ADAM-TS 13; ADAM-TS13; ADAMTS-13; vWF-CP]

ATS13_HUMAN

>90%, as determined by SDS-PAGE and visualized by silver stain.

Supplied as a liquid in 25mM HEPES, 300mM sodium chloride, pH 8.0

0.33 mg/ml

Aliquot to avoid repeated freezing and thawing and store at -70°C. Aliquots are stable for 6 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Shipping: Dry Ice.

N-terminal Sequence Analysis: Ala75. Biological Activity: Measured by its ability to cleave the fluorogenic peptide substrate, FRETS-VWF73. The specific activity is >10pmol/min/ug. Grade: Purified. Endotoxin: < 1 EU/ug (LAL)

Activity Assay Protocol: Materials:. -Assay Buffer (TCNB): 50mM Tris, 2 mM CaCl2, 0.05% Brij35, pH 5.5. -MBS636953: Recombinant human ADAMTS13 . -Substrate: FRETS-VWF73, 100uM in DMSO. -F16 Black Maxisorp Plate. -Fluorescent Plate Reader. Assay:. 1. Dilute MBS636953 to 5ug/ml in assay buffer. 2. Dilute substrate to 8uM in assay buffer. 3. Load 50ul of dilute MBS636953 into a plate, and start the reaction by adding 50ul of 8uM substrate. Include a Substrate Blank containing 50ul of Assay Buffer and 50ul of 8ul substrate. 4. Read at excitation and emission wavelengths of 340nm and 450 nm (top read), respectively, in kinetic mode for 5 minutes. 5. Calculate specific activity:. Sp Act (pmol/min/ug)= *Adjusted Vmax (RFU/min) x **Conversion Factor (pmol/RFU) / amount of enzyme (ug) . *Adjusted for substrate blank. **Derived using calibration standard FRETS25STD1. Final Assay Conditions: rhADAMTS13: 0.25ug

Molecular Biology; MB-Matrix Metalloproteinases

ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin motifs 13), also known as von Willebran Factor (vWF) cleaving protease, is a member of the family of secreted zinc proteases with a multi-domain structure (1-3). The protein precursors consist of a signal peptide and following domains: pro, catalytic, disintegrin-like, TS type 1 motif, cystein-rich, spacer, a second set of seven TSP1 repeats, and two CUB domins. The only known substrate of ADAMTS13 is vWF, a blood glycoprotein with two homeostatic functions (4). It is required for platelet adhesion to sites of vascular damage and acts as a carrier protein for blood-clotting factor VIII in the circulation. It exists in plasma as multimers, the largest of which effectively mediate platelet adhesion. ADAMTS13 cleaves multimeric vWF in the A2 domain at the position, Tyr 1605-Met 1606. Defect in ADAMTS13 activity is a cause of congenital thrombotic thrombocytopenic purpura (TTP), also known as Upshaw-Schulman syndrome. Lack of ADAMTS13 activity allows unusually large vWF (UlvWF) to occur in plasma (5). These UlvWF multimers have tendency to agglutinate circulating platelets at sites with high levels of shear stress to cause TTP. Recombinant protein corresponding to Gln34-Trp688 with a C-terminal 10X-His tag from human ADAMTS13 (Accession #NP_620594), expressed in CHO cell line. The purified rhADAMTS13 starts at the N-terminus of the pro domain and ends in the spacer domain.

73695936

NP_620596.2

NM_139027.4

Q76LX8

Predicted: 73kD. Observed: 90kD (reducing conditions)

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: Protease; EC 3.4.24.87; Extracellular matrix; Secreted, signal peptide; Calcium-binding; Motility/polarity/chemotaxis; 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; 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; response to toxin; cell-matrix adhesion; peptide catabolic process; protein processing; proteolysis; post-translational protein modification. Disease: Thrombotic Thrombocytopenic Purpura, Congenital

0.02 mg

740 USD