Rabbit Anti-ADAMTS13 antibody
Cleaves the vWF multimers in plasma into smaller forms. Von Willebrand factor cleaving protease; A disintegrin and metalloproteinase with thrombospondin motifs 13; A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif 1
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Product Name ADAMTS13 Chinese Name ADAMTS13Recombinant rabbit monoclonal anti Alias Cleaves the vWF multimers in plasma into smaller forms. Von Willebrand factor cleaving protease; A disintegrin and metalloproteinase with thrombospondin motifs 13; A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif 13; A disintegrin like and metalloprotease with thrombospondin type 1 motif 13; ADAM metallopeptidase with thrombospondin type 1 motif 13; ADAM TS 13; ADAM TS13; ADAM-TS 13; ADAM-TS13; ADAMTS 13; ADAMTS-13; ADAMTS13; ADAMTS13 protein; ATS13_HUMAN; C9orf8; TTP; von Willebrand factor-cleaving protease; vWF cleaving protease; vWF CP; vWF-cleaving protease; vWF-CP; vWFCP. 整合素样金属蛋白酶与凝血酶13型抗体 Research Area Tumour Cell biology immunology Signal transduction Cell adhesion molecule Immunogen Species Rabbit Clonality Monoclonal Clone NO. 4G7 React Species Human, Applications WB=1:500-2000 IHC-P=1:20-200 IHC-F=1:20-200 Flow-Cyt=1:50 （Paraffin sections need antigen repair）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 145kDa Cellular localization Secretory protein Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human ADAMTS13 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail This gene encodes a member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motif) protein family. Members of the family share several distinct protein modules, including a propeptide region, a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 (TS) motif. Individual members of this family differ in the number of C-terminal TS motifs, and some have unique C-terminal domains. The enzyme encoded by this gene is the von Willebrand Factor (vWF)-cleaving protease, which is responsible for cleaving at the site of Tyr842-Met843 of the vWF molecule. A deficiency of this enzyme is associated with thrombotic thrombocytopenic purpura. Alternative splicing of this gene generates multiple transcript variants encoding different isoforms. [provided by RefSeq, Nov 2008].
Function:
Cleaves the vWF multimers in plasma into smaller forms.
Subcellular Location:
Secreted. Note=Secretion enhanced by O-fucosylation of TSP type-1 repeats.
Tissue Specificity:
Plasma. Expressed primarily in liver.
Post-translational modifications:
Glycosylated. O-fucosylated by POFUT2 on a serine or a threonine residue found within the consensus sequence C1-X(2)-(S/T)-C2-G of the TSP type-1 repeat domains where C1 and C2 are the first and second cysteine residue of the repeat, respectively. Fucosylated repeats can then be further glycosylated by the addition of a beta-1,3-glucose residue by the glucosyltransferase, B3GALTL. Fucosylation mediates the efficient secretion of ADAMTS13. May also be C-glycosylated on tryptophan residues within the consensus sequence W-X-X-W of the TPRs, and also N-glycosylated. These other glycosylations can also facilitate secretion.
The precursor is processed by a furin endopeptidase which cleaves off the pro-domain.
DISEASE:
Defects in ADAMTS13 are the cause of thrombotic thrombocytopenic purpura congenital (TTP) [MIM:274150]; 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.
Similarity:
Contains 2 CUB domains.
Contains 1 disintegrin domain.
Contains 1 peptidase M12B domain.
Contains 8 TSP type-1 domains.
SWISS:
Q76LX8
Gene ID:
11093
Database links:Entrez Gene: 11093 Human
Entrez Gene: 279028 Mouse
Omim: 604134 Human
SwissProt: Q76LX8 Human
SwissProt: Q769J6 Mouse
Unigene: 131433 Human
Unigene: 330084 Mouse
Product Picture 
Sample:
Lane 1: SiHa cell lysate
Lane 2: PC-3M cell lysate
Lane 3: A549 cell lysate
Primary: Anti-ADAMTS13 (SLM-54417R) at 1:500 dilution
Secondary: Goat Anti-Rabbit IgG - HRP at 1:5000 dilution
Predicted band size: 145 kD
Observed band size: 250 kD
Paraformaldehyde-fixed, paraffin embedded (human liver); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ADAMTS13) Monoclonal Antibody, Unconjugated (SLM-54417R) at 1:50 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (human colon); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ADAMTS13) Monoclonal Antibody, Unconjugated (SLM-54417R) at 1:50 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Blank control:HepG2.
Primary Antibody (green line): Rabbit Anti-ADAMTS13 antibody (bms-54417R)
Dilution: 1:50;
Isotype Control Antibody (orange line): Rabbit IgG .
Secondary Antibody : Goat anti-rabbit IgG-AF488
Dilution: 1:1000.
Protocol
The cells were fixed with 4% PFA (10min at room temperature)and then permeabilized with 0.1% PBST for 20 min at room temperature.The cells were then incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.
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