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GW21023

Sigma-Aldrich

Anti-ATP7A antibody produced in chicken

affinity isolated antibody, buffered aqueous solution

Sinónimos:

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

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About This Item

MDL number:
MFCD03455449
UNSPSC Code:
12352203
NACRES:
NA.41

biological source

chicken

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

species reactivity

human, mouse, rat

manufacturer/tradename

Genway 15-288-21023

technique(s)

western blot: suitable

NCBI accession no.

NP_000043.1

UniProt accession no.

Q04656

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... ATP7A(538)

Immunogen

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.

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Storage Class

10 - Combustible liquids

wgk_germany

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

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

Certificados de análisis (COA)

Busque Certificados de análisis (COA) introduciendo el número de lote del producto. Los números de lote se encuentran en la etiqueta del producto después de las palabras «Lot» o «Batch»

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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
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
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

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