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Key Documents

SAB1303565

Sigma-Aldrich

ANTI-ADAMTS13 (CENTER) antibody produced in rabbit

IgG fraction of antiserum, buffered aqueous solution

Synonyme(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

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About This Item

Code UNSPSC :
12352203
Nomenclature NACRES :
NA.41

Source biologique

rabbit

Niveau de qualité

100

Conjugué

unconjugated

Forme d'anticorps

IgG fraction of antiserum

Type de produit anticorps

primary antibodies

Clone

polyclonal

Forme

buffered aqueous solution

Poids mol.

153604 Da

Espèces réactives

human

Technique(s)

immunohistochemistry: 1:50-1:100
western blot: 1:250-1:500

Numéro d'accès UniProt

Q76LX8

Conditions d'expédition

wet ice

Température de stockage

−20°C

Modification post-traductionnelle de la cible

unmodified

Informations sur le gène

human ... ADAMTS13(11093)

Description générale

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.

Actions biochimiques/physiologiques

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.

Forme physique

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.

Clause de non-responsabilité

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.

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Code de la classe de stockage

10 - Combustible liquids

Classe de danger pour l'eau (WGK)

nwg

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable

Certificats d'analyse (COA)

Recherchez un Certificats d'analyse (COA) en saisissant le numéro de lot du produit. Les numéros de lot figurent sur l'étiquette du produit après les mots "Lot" ou "Batch".

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Consulter la Bibliothèque de documents

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)
Zinc and calcium ions cooperatively modulate ADAMTS13 activity
Anderson PJ, et al.
The Journal of Biological Chemistry, 281(2), 850-857 (2006)
A functional calcium-binding site in the metalloprotease domain of ADAMTS13
Gardner MD, et al.
Blood, 113(5), 1149-1157 (2009)
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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