SAB1303565
ANTI-ADAMTS13 (CENTER) antibody produced in rabbit
IgG fraction of antiserum, buffered aqueous solution
别名:
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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所有图片(1)
About This Item
分類程式碼代碼:
12352203
NACRES:
NA.41
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生物源
rabbit
品質等級
共軛
unconjugated
抗體表格
IgG fraction of antiserum
抗體產品種類
primary antibodies
無性繁殖
polyclonal
形狀
buffered aqueous solution
分子量
153604 Da
物種活性
human
技術
immunohistochemistry: 1:50-1:100
western blot: 1:250-1:500
UniProt登錄號
運輸包裝
wet ice
儲存溫度
−20°C
目標翻譯後修改
unmodified
基因資訊
human ... ADAMTS13(11093)
一般說明
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.
生化／生理作用
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.
外觀
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.
免責聲明
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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儲存類別代碼
10 - Combustible liquids
水污染物質分類（WGK）
nwg
閃點（°F）
Not applicable
閃點（°C）
Not applicable
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)
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)
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