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Merck

GW21023

Sigma-Aldrich

Anti-ATP7A antibody produced in chicken

affinity isolated antibody, buffered aqueous solution

别名:

Anti-ATPase, Cu++ transporting, α polypeptide, Anti-Copper-transporting ATPase 1, Anti-Menkes disease-associated protein

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About This Item

MDL號碼:
分類程式碼代碼:
12352203
NACRES:
NA.41

生物源

chicken

品質等級

共軛

unconjugated

抗體表格

affinity isolated antibody

抗體產品種類

primary antibodies

無性繁殖

polyclonal

形狀

buffered aqueous solution

物種活性

human, mouse, rat

製造商/商標名

Genway 15-288-21023

技術

western blot: suitable

NCBI登錄號

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 ... ATP7A(538)

免疫原

Immunogen Sequence: GI # 4502321, sequence 1407-1500
Recombinant ATPase

應用

Anti-ATP7A antibody produced in chicken is suitable for western blotting analysis at a dilution of 1:500, for tissue or cell staining at a dilution of 1:200.

生化/生理作用

Copper-transporting ATPase 1 is a protein encoded by the ATP7A gene in humans. It mediates systemic copper absorption and provides cupro-enzymes in the trans-Golgi network (TGN) with copper. It constitutively cycles between the TGN and plasma membrane (PM) for regulating the metal homeostasis. Mutation in this gene causes Menkes disease, a rare X-linked recessive disorder characterized by multi-systemic disorder of copper deficiency. This disease is caused due to a novel "silent" substitution in the ATP7A gene.

外觀

Solution in phosphate buffered saline containing 0.02% sodium azide.

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)

WGK 1

閃點(°F)

Not applicable

閃點(°C)

Not applicable

個人防護裝備

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


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Lisbeth Birk Møller et al.
Molecular genetics and metabolism, 110(4), 490-492 (2013-10-09)
We present a case of classical Menkes disease (MD) due to a novel "silent" substitution in the ATP7A gene; c.2781G>A (p.K927K). The affected nucleotide is the last nucleotide in exon 13, and affects mRNA splicing. Transcripts missing exon 13; and
Qiao Wang et al.
Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics, 16(6), 624-628 (2014-06-14)
Menkes disease is a rare X-linked recessive disorder characterized by multi-systemic disorder of copper deficiency caused by ATP7A gene mutation. In this study, the clinical and laboratory features of three patients with Menkes disease were analyzed. Prenatal diagnosis had been
Varadarajan Sudhahar et al.
American journal of physiology. Cell physiology, 319(5), C933-C944 (2020-09-17)
Caveolin-1 (Cav-1) is a scaffolding protein and a major component of caveolae/lipid rafts. Previous reports have shown that endothelial dysfunction in Cav-1-deficient (Cav-1-/-) mice is mediated by elevated oxidative stress through endothelial nitric oxide synthase (eNOS) uncoupling and increased NADPH
Zoe G Holloway et al.
Molecular biology of the cell, 24(11), 1735-1748 (2013-04-19)
The transporter ATP7A mediates systemic copper absorption and provides cuproenzymes in the trans-Golgi network (TGN) with copper. To regulate metal homeostasis, ATP7A constitutively cycles between the TGN and plasma membrane (PM). ATP7A trafficking to the PM is elevated in response

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