生物来源
chicken
质量水平
偶联物
unconjugated
抗体形式
affinity isolated antibody
抗体产品类型
primary antibodies
克隆
polyclonal
形式
buffered aqueous solution
种属反应性
human, mouse, rat
制造商/商品名称
Genway 15-288-21023
技术
western blot: suitable
NCBI登记号
UniProt登记号
运输
wet ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... ATP7A(538)
应用
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.
外形
Solution in phosphate buffered saline containing 0.02% sodium azide.
免责声明
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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WGK
WGK 1
闪点(°F)
Not applicable
闪点(°C)
Not applicable
个人防护装备
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
法规信息
新产品
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
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
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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