Immunogen Sequence: GI # 4502321, sequence 1407-1500
Recombinant ATPase
Application
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.
Biochem/physiol Actions
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.
Physical form
Solution in phosphate buffered saline containing 0.02% sodium azide.
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.
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
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
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
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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