SAB1303565
ANTI-ADAMTS13 (CENTER) antibody produced in rabbit
IgG fraction of antiserum, buffered aqueous solution
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Synonym(s):
A disintegrin and metalloproteinase with thrombospondin motifs 13, ADAM-TS 13, ADAM-TS13, ADAMTS-13, ADAMTS13, C9orf8, vWF-CP, vWF-cleaving protease, von Willebrand factor-cleaving protease
UNSPSC Code:
12352203
NACRES:
NA.41
Recommended Products
biological source
rabbit
Quality Level
conjugate
unconjugated
antibody form
IgG fraction of antiserum
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
153604 Da
species reactivity
human
technique(s)
immunohistochemistry: 1:50-1:100
western blot: 1:250-1:500
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... ADAMTS13(11093)
General description
A disintegrin-like and metalloprotease with thrombospondin type 1 motif, 13 (ADAMTS13) also known as von willebrand factor-cleaving protease (VWFCP), has distinct regions like a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 domain and spacer domain rich in cysteine residues. ADAMST13 is highly expressed in the liver and secreted as an active enzyme in the blood. In human chromosome, the gene ADAMTS13 is localised on 9q34.
Biochem/physiol Actions
A disintegrin-like and metalloprotease with thrombospondin type 13 (ADAMTS13) cleaves von Willebrand factor (VWF) and thereby controls platelet thrombus formation. ADAMTS13 requires zinc, and calcium for its effective catalytic activity. Mutations in ADAMTS13 can lead to life threatening acquired thrombotic thrombocytopenic purpura (TTP) or hemolytic-uremic syndrome. Congenital deficiency of ADAMTS13 leads to Upshaw−Schulman syndrome.
Physical form
Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
WGK
nwg
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Regulatory Information
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Certificates of Analysis (COA)
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Novel compound heterozygote mutations (H234Q/R1206X) of the ADAMTS13 gene in an adult patient with Upshaw-Schulman syndrome showing predominant episodes of repeated acute renal failure
Shibagaki Y, et al.
Nephrology, Dialysis, and Transplantation, 21(5), 1289-1292 (2006)
A functional calcium-binding site in the metalloprotease domain of ADAMTS13
Gardner MD, et al.
Blood, 113(5), 1149-1157 (2009)
Zinc and calcium ions cooperatively modulate ADAMTS13 activity
Anderson PJ, et al.
The Journal of Biological Chemistry, 281(2), 850-857 (2006)
Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura
Levy GG, et al.
Nature, 413(6855), 488-488 (2001)
Crystal structures of the noncatalytic domains of ADAMTS13 reveal multiple discontinuous exosites for von Willebrand factor
Akiyama M, et al.
Proceedings of the National Academy of Sciences of the USA, 106(46), 19274-19279 (2009)
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