ADAMTS13 Antibody (PACO57652)
- SKU:
- PACO57652
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- IHC
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Frequently bought together:
Description
| Antibody Name: | ADAMTS13 Antibody (PACO57652) |
| Antibody SKU: | PACO57652 |
| Size: | 50ug |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, IHC |
| Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:200-1:500 |
| Species Reactivity: | Human |
| Immunogen: | Recombinant Human A disintegrin and metalloproteinase with thrombospondin motifs 13 protein (738-905AA) |
| Form: | Liquid |
| Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
| Purification Method: | >95%, Protein G purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
 | IHC image of PACO57652 diluted at 1:400 and staining in paraffin-embedded human liver tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
| Background: | Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation. |
| Synonyms: | 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), ADAMTS13, C9orf8 |
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | Protein type:Motility/polarity/chemotaxis; Calcium-binding; Secreted, signal peptide; Secreted; Protease; EC 3.4.24.87; Extracellular matrix 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 |
| NCBI Summary: | 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 Code: | Q76LX8 |
| NCBI GenInfo Identifier: | 74749836 |
| NCBI Gene ID: | 11093 |
| NCBI Accession: | Q76LX8.1 |
| UniProt Secondary Accession: | Q76LX8,Q6UY16, Q710F6, Q711T8, Q96L37, Q9H0G3, Q9UGQ1 |
| UniProt Related Accession: | Q76LX8 |
| Molecular Weight: | 39,864 Da |
| NCBI Full Name: | A disintegrin and metalloproteinase with thrombospondin motifs 13 |
| NCBI 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 |
| UniProt Synonym Protein Names: | von Willebrand factor-cleaving protease; vWF-CP; vWF-cleaving protease |
| Protein Family: | A disintegrin and metalloproteinase with thrombospondin motifs |
| UniProt Gene Name: | ADAMTS13 |
| UniProt Entry Name: | ATS13_HUMAN |
| Secondary Antibody |
| Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
| Recommended Products |
| Anti-FITC Goat Anti-Rabbit IgG (H+L) Antibody (CABS011) |
| Anti-HRP-conjugated Beta Actin Antibody (CABC028) |
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