Human ADAMTS13 ELISA Kit | ADAMTS13 elisa kit

Human ADAMTS13 ELISA Kit

Cat. Num. AAA8248328

• Gene Names: ADAMTS13; VWFCP; C9orf8; vWF-CP; ADAM-TS13; ADAMTS-13
• Reactivity: Human
• Synonyms: ADAMTS13; Human ADAMTS13 ELISA Kit; C9orf8; A disintegrin and metalloproteinase with thrombospondin motifs 13; ADAM-TS 13; ADAM-TS13; ADAMTS-13; von Willebrand factor-cleaving protease; vWF-CP; vWF-cleaving protease; ADAMTS13 elisa kit

• Reactivity: Human
• Specificity: The Human ADAMTS13 ELISA Kit allows for the detection and quantification of endogenous levels of natural and/or recombinant Human ADAMTS13 proteins within the range of 780 pg/ml - 50000 pg/ml.
• Sequence Length: 1427

• Samples: Cell culture supernates, serum, plasma
• Sensitivity: < 20 pg/ml
• Preparation and Storage: Shipped and store at 4 degree C for 6 months, store at -20 degree C for one year. Avoid freeze/thaw cycles.

Typical Testing Data/Standard Curve (for reference only)

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Related Product Information for ADAMTS13 elisa kit

Principle of the Assay: The Human ADAMTS13 ELISA (Enzyme-Linked Immunosorbent Assay) kit is an in vitro enzyme-linked immunosorbent assay for the quantitative measurement of Human ADAMTS13 in Cell Culture Supernates, Serum, Plasma. This assay employs an antibody specific for Human ADAMTS13 coated on a 96-well plate. Standards and samples are pipetted into the wells and ADAMTS13 present in a sample is bound to the wells by the immobilized antibody. The wells are washed and biotinylated anti-Human ADAMTS13 antibody is added. After washing away unbound biotinylated antibody, HRP-conjugated streptavidin is pipetted to the wells. The wells are again washed, a TMB substrate solution is added to the wells and color develops in proportion to the amount of ADAMTS13 bound. The Stop Solution changes the color from blue to yellow, and the intensity of the color is measured at 450 nm.

Background/Introduction: ADAMTS13 is a zinc-containing metalloprotease enzyme. The ADAMTS13 gene is mapped to chromosome 9q34 by genomic sequence analysis. It is reported that a metal-containing proteolytic enzyme (metalloprotease) in normal plasma cleaves the peptide bond between tyrosine at position 842 and methionine at position 843 in monomeric subunits of von Willebrand factor, thereby degrading the large multimers. And it is confirmed that the ADAMTS13 gene encodes the von Willebrand factor-cleaving protease (VWFCP). ADAMTS13 is secreted in blood and degrades large vWf multimers, decreasing their activity.

NCBI and Uniprot Product Information

• NCBI GI #: 21265034
• NCBI GeneID: 11093
• NCBI Accession #: NP_620594.1
• NCBI GenBank Nucleotide #: NM_139025.4
• UniProt Accession #: Q76LX8; Q6UY16; Q710F6; Q711T8; Q96L37; Q9H0G3; Q9UGQ1
• Molecular Weight: 39,864 Da
• NCBI Official Full Name: A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform 1 preproprotein
• NCBI Official Synonym Full Names: ADAM metallopeptidase with thrombospondin type 1 motif 13
• NCBI Official Symbol: ADAMTS13
• NCBI Official Synonym Symbols: VWFCP; C9orf8; vWF-CP; ADAM-TS13; ADAMTS-13
• NCBI Protein Information: A disintegrin and metalloproteinase with thrombospondin motifs 13
• UniProt Protein Name: A disintegrin and metalloproteinase with thrombospondin motifs 13
• Protein Family: A disintegrin and metalloproteinase with thrombospondin motifs
• UniProt Gene Name: ADAMTS13
• UniProt Synonym Gene Names: C9orf8; ADAM-TS 13; ADAM-TS13; ADAMTS-13; vWF-CP; vWF-cleaving protease
• UniProt Entry Name: ATS13_HUMAN

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; Extracellular matrix; Motility/polarity/chemotaxis; Protease; Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 9q34

Cellular Component: endoplasmic reticulum lumen; proteinaceous extracellular matrix

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

Biological Process: peptide catabolic process; protein processing; proteolysis

Disease: Thrombotic Thrombocytopenic Purpura, Congenital