Key features and details
- Rabbit polyclonal to ADAMTS13
- Suitable for: ICC/IF, WB
- Reacts with: Human
- Isotype: IgG
Product nameAnti-ADAMTS13 antibody
See all ADAMTS13 primary antibodies
DescriptionRabbit polyclonal to ADAMTS13
Specificityab28273 recognises the Carboxyterminal end of the long form of ADAMTS13.
Tested applicationsSuitable for: ICC/IF, WBmore details
Species reactivityReacts with: Human
Synthetic peptide based on the carboxyterminal end of full length human ADAMTS13. (Peptide available as ab41251.)
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term.
Storage bufferpH: 7.40
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab28273 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||Use a concentration of 1 µg/ml.|
|WB||1/1000 - 1/5000. Detects a band of approximately 190 kDa (predicted molecular weight: 154 kDa). 1/1000, when using colorimetric substrates such as BCIP/NBT - 1/5000, when using chemiluminescent substrates. Detects a band of approximately 190 kDa. Glycosylation and the abundance of cysteine residues gives ADAMTS13 an apparent molecular weight of 190 kDa on reduced SDS PAGE gels. Several bands between 110-190 kDa are observed on Western blots, possibly indicating differentially processed ADAMTS 13. Dilution optimised using Chromogenic detection.|
FunctionCleaves the vWF multimers in plasma into smaller forms.
Tissue specificityPlasma. Expressed primarily in liver.
Involvement in diseaseDefects 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.
Sequence similaritiesContains 2 CUB domains.
Contains 1 disintegrin domain.
Contains 1 peptidase M12B domain.
Contains 8 TSP type-1 domains.
DomainThe pro-domain is not required for folding or secretion and does not perform the common function of maintening enzyme latency.
The spacer domain is necessary to recognize and cleave vWF. The C-terminal TSP type-1 and CUB domains may modulate this interaction.
modificationsMay contain a C-mannosylation site and O-fucosylation sites in the TSP type-1 domains.
The precursor is processed by a furin endopeptidase which cleaves off the pro-domain.
- Information by UniProt
- A disintegrin and metalloproteinase with thrombospondin motifs 13 antibody
- A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif 13 antibody
- A disintegrin like and metalloprotease with thrombospondin type 1 motif 13 antibody
All lanes : Anti-ADAMTS13 antibody (ab28273) at 1 µg/ml
Lane 1 : Recombinant Human ATS-13 at 0.08 µg
Lane 2 : Recombinant Human ATS-13 at 0.04 µg
Lane 3 : Recombinant Human ATS-13 at 0.02 µg
Predicted band size: 154 kDa
Glycosylation and the abundance of cysteine residues gives ADAMTS-13 an apparent molecular weight of about 190 kDa on reduced SDS PAGE gels.
ICC/IF image of ab28273 stained HepG2 cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab28273, 1µg/ml) overnight at +4°C. The secondary antibody (green) was ab96899, DyLight® 488 goat anti-rabbit IgG (H+L) used at a 1/250 dilution for 1h.Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.
ab28273 has not yet been referenced specifically in any publications.