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Merck
모든 사진(1)

Key Documents

HPA012887

Sigma-Aldrich

Anti-ATP7A antibody produced in rabbit

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

동의어(들):

Anti-Copper pump 1, Anti-Copper-transporting ATPase 1, Anti-Menkes disease-associated protein

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About This Item

UNSPSC 코드:
12352203
인간 단백질 도해서 번호:
NACRES:
NA.43

생물학적 소스

rabbit

Quality Level

결합

unconjugated

항체 형태

affinity isolated antibody

항체 생산 유형

primary antibodies

클론

polyclonal

제품 라인

Prestige Antibodies® Powered by Atlas Antibodies

형태

buffered aqueous glycerol solution

종 반응성

human

기술

immunohistochemistry: 1:20- 1:50

면역원 서열

TETLGTCIDFQVVPGCGISCKVTNIEGLLHKNNWNIEDNNIKNASLVQIDASNEQSSTSSSMIIDAQISNALNAQQYKVLIGNREWMIRNGLVINNDVNDFMTEHERKGRTAVLVAVDDELC

UniProt 수납 번호

배송 상태

wet ice

저장 온도

−20°C

타겟 번역 후 변형

unmodified

유전자 정보

human ... ATP7A(538)

일반 설명

Copper-transporting ATPase 1 (ATP7A) is a P-type ATPase localized in the trans-Golgi network (TGN). It has an amino-terminal region with six metal binding motifs, eight transmembrane domains and several other residues or motifs generally present in heavy metal-transporting ATPases. The gene encoding this protein is localized on chromosome Xq13.3.

면역원

Copper-transporting ATPase 1 recombinant protein epitope signature tag (PrEST)

애플리케이션

All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.

The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.

생화학적/생리학적 작용

Copper-transporting ATPase 1 (ATP7A) is mainly involved in delivering copper to copper-requiring enzymes. At times when cells are exposed to excessive copper, it is rapidly relocalized from the trans-Golgi network (TGN) to the plasma membrane and takes care of copper efflux. Thus, its functions include the transport of copper to cuproenzymes and proteins in the secretory pathway as well as excretion of excess copper. It constantly cycles between the TGN and plasma membrane. Mutations in the gene encoding ATP7A have been linked to Menkes disease.

특징 및 장점

Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:
  • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
  • Protein array of 364 human recombinant protein fragments.

결합

Corresponding Antigen APREST74406

물리적 형태

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

법적 정보

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

면책조항

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 Code

10 - Combustible liquids

WGK

WGK 1

Flash Point (°F)

Not applicable

Flash Point (°C)

Not applicable

개인 보호 장비

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


시험 성적서(COA)

제품의 로트/배치 번호를 입력하여 시험 성적서(COA)을 검색하십시오. 로트 및 배치 번호는 제품 라벨에 있는 ‘로트’ 또는 ‘배치’라는 용어 뒤에서 찾을 수 있습니다.

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문서 라이브러리 방문

C Vulpe et al.
Nature genetics, 3(1), 7-13 (1993-01-01)
Menkes disease is an X-linked disorder of copper transport characterized by progressive neurological degeneration and death in early childhood. We have isolated a candidate gene (Mc1) for Menkes disease and find qualitative or quantitative abnormalities in the mRNA in sixteen
Sarah E M Stephenson et al.
The Journal of biological chemistry, 280(39), 33270-33279 (2005-07-30)
The homeostatic regulation of essential elements such as copper requires many proteins whose activities are often mediated and tightly coordinated through protein-protein interactions. This regulation ensures that cells receive enough copper without intracellular concentrations reaching toxic levels. To date, only
M J Petris et al.
Human molecular genetics, 8(11), 2107-2115 (1999-09-15)
Menkes disease is an X-linked recessive copper deficiency disorder caused by mutations in the ATP7A ( MNK ) gene which encodes a copper transporting P-type ATPase (MNK). MNK is normally localized pre- dominantly in the trans -Golgi network (TGN); however
M Qi et al.
Human molecular genetics, 7(3), 465-469 (1998-04-18)
The ATP7A gene encodes a copper-transporting ATPase. Mutations in this gene result in two clinically distinct X-linked inherited disorders: Menkes disease and occipital horn syndrome (OHS). We identified a single exon skipping in the ATP7A transcript in cells from the
Z Tümer et al.
Advances in experimental medicine and biology, 448, 83-95 (1999-03-18)
Our knowledge about Menkes disease (MD) has expanded greatly since its description in 1962 as a new X-linked recessive neurodegenerative disorder of early infancy. Ten years later a defect in copper metabolism was established as the underlying biochemical deficiency. In

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