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ATP7A Antibody – Rabbit Polyclonal

For Research Use Only. Not for use in diagnostic procedures. Catalog #: MBS1758150

The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring… More details

$210 / 0.01 mg +1 FREE 8GB USB
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Product Overview

Online Datasheet in PDF
Product Name
ATP7A, Polyclonal Antibody
Full Product Name
Anti-ATP7A Antibody Picoband
Product Gene Name
anti-ATP7A antibody
Product Synonym Names
Copper-transporting ATPase 1; ATPase copper transporting alpha; ATP7A; MC1; MNK; Copper-transporting ATPase 1; EC 7.2.2.8; Copper pump 1; Menkes disease-associated protein
Clonality
Polyclonal
Isotype
Rabbit IgG
Host
Rabbit
Species Reactivity
Human
Form/Format
Lyophilized. Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
Purity/Purification
Immunogen affinity purified.
Preparation and Storage
At -20 degree C for one year from date of receipt. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for six months.Avoid repeated freezing and thawing.
Molecular Weight
11,522 Da
OMIM
phenotype 309400
3D Structure
ModBase 3D Structure for Q04656
Immunogen
A synthetic peptide corresponding to a sequence at the C-terminus of human ATP7A, which shares 79.2% amino acid (aa) sequence identity with both mouse and rat ATP7A.
Reconstitution
Adding 0.2 ml of distilled water will yield a concentration of 500ug/ml.
Cross Reactivity
No cross-reactivity with other proteins

Product Categories/Family for anti-ATP7A antibody

Primary Antibodies
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Applications Tested/Suitable for anti-ATP7A antibody
Western Blot (WB), Flow Cytometry (FC/FACS)
Application Notes for anti-ATP7A antibody
WB: 0.25-0.5ug/ml, Human
FC/FACS (Fixed): 1-3ug/1x106 cells, Human

Western Blot (WB) of anti-ATP7A antibody

Figure 1. Western blot analysis of ATP7A using anti-ATP7A antibody (MBS1758150).
Electrophoresis was performed on a 5-20% SDS-PAGE gel at 70V (Stacking gel)/90V (Resolving gel) for 2-3 hours. The sample well of each lane was loaded with 30 ug of sample under reducing conditions.
Lane 1: human HepG2 whole cell lysates,
Lane 2: human SH-SY5Y whole cell lysates,
Lane 3: human K562 whole cell lysates.
After electrophoresis, proteins were transferred to a nitrocellulose membrane at 150 mA for 50-90 minutes. Blocked the membrane with 5% non-fat milk/TBS for 1.5 hour at RT. The membrane was incubated with rabbit anti-ATP7A antigen affinity purified polyclonal antibody (#MBS1758150) at 0.5ug/mL overnight at 4 degree C, then washed with TBS-0.1%Tween 3 times with 5 minutes each and probed with a goat anti-rabbit IgG-HRP secondary antibody at a dilution of 1:5000 for 1.5 hour at RT. The signal is developed using an Enhanced Chemiluminescent detection (ECL) kit (#MBS176460) with Tanon 5200 system. A specific band was detected for ATP7A at approximately 163 kDa. The expected band size for ATP7A is at 163 kDa.
Rabbit Anti-ATP7A Polyclonal Antibody – Cat# MBS1758150

Flow Cytometry (FC/FACS) of anti-ATP7A antibody

Figure 2. Flow Cytometry analysis of HepG2 cells using anti-ATP7A antibody (MBS1758150).
Overlay histogram showing HepG2 cells stained with MBS1758150 (Blue line). The cells were fixed with 4% paraformaldehyde and blocked with 10% normal goat serum. And then incubated with rabbit anti-ATP7A Antibody (MBS1758150, 1ug/1x106 cells) for 30 min at 20 degree C. DyLight488 conjugated goat anti-rabbit IgG (MBS177405, 5-10ug/1x106 cells) was used as secondary antibody for 30 minutes at 20 degree C. Isotype control antibody (Green line) was rabbit IgG (1ug/1x106) used under the same conditions. Unlabelled sample without incubation with primary antibody and secondary antibody (Red line) was used as a blank control.
Rabbit Anti-ATP7A Polyclonal Antibody, Flow Cytometry (FC/FACS) – Cat# MBS1758150

Product References and Citations for anti-ATP7A antibody

1. Cecchi, C., Biasotto, M., Tosi, M., Avner, P. The mottled mouse as a model for human Menkes disease: identification of mutations in the Atp7a gene. Hum. Molec. Genet. 6: 425-433, 1997. Note: Erratum: Hum. Molec. Genet. 6: 829 only, 1997.2. Chelly, J., Tumer, Z., Tonnesen, T., Petterson, A., Ishikawa-Brush, Y., Tommerup, N., Horn, N., Monaco, A. P. Isolation of a candidate gene for Menkes disease that encodes a potential heavy metal binding protein. Nature Genet. 3: 14-19, 1993.3. Christodoulou, J., Danks, D. M., Sarkar, B., Baerlocher, K. E., Casey, R., Horn, N., Tumer, Z., Clarke, J. T. R. Early treatment of Menkes disease with parenteral cooper (sic)-histidine: long-term follow-up of four treated patients. Am. J. Med. Genet. 76: 154-164, 1998.

Other Notes

Small volumes of anti-ATP7A antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Searchable Terms for ATP7A purchase

MBS1758150 is an antibody targeting ATP7A. Also known as Anti-ATP7A Antibody Picoband. Reactive in Human. Host: Rabbit.

Additional Details

NCBI/Uniprot data below describe general gene information for ATP7A. It may not necessarily be applicable to this product.

NCBI GI #
115529486
NCBI GeneID
538
NCBI Accession #
NP_000043.3
UniProt Primary Accession #
Q04656
UniProt Secondary Accession #
O00227; O00745; Q9BYY8; B1AT72
UniProt Related Accession #
Q04656
GenBank Nucleotide #
NM_000052.5
NCBI Official Full Name
copper-transporting ATPase 1
NCBI Official Synonym Full Names
ATPase, Cu++ transporting, alpha polypeptide
NCBI Official Symbol
ATP7A
NCBI Official Synonym Symbols
MK; MNK; DSMAX; SMAX3
UniProt Protein Name
Copper-transporting ATPase 1
UniProt Synonym Protein Names
Copper pump 1; Menkes disease-associated protein
Protein Family
Peroxidase
UniProt Gene Name
ATP7A
UniProt Synonym Gene Names
MC1; MNK
UniProt Entry Name
ATP7A_HUMAN
NCBI Protein Information
copper-transporting ATPase 1; copper pump 1; Cu++-transporting P-type ATPase; Menkes disease-associated protein

NCBI Summary for ATP7A

This gene encodes a transmembrane protein that functions in copper transport across membranes. This protein is localized to the trans Golgi network, where it is predicted to supply copper to copper-dependent enzymes in the secretory pathway. It relocalizes to the plasma membrane under conditions of elevated extracellular copper, and functions in the efflux of copper from cells. Mutations in this gene are associated with Menkes disease, X-linked distal spinal muscular atrophy, and occipital horn syndrome. Alternatively-spliced transcript variants have been observed. [provided by RefSeq, Aug 2013]
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UniProt Comments for ATP7A

ATP7A: a multi-pass membrane protein and cation transporting ATPase. A copper-transporter that may supply copper to copper-requiring proteins within the secretory pathway when localized in the trans-Golgi network. Cycles between the trans-Golgi network (TGN) and the plasma membrane. Predominantly found in the TGN and relocalizes to the plasma membrane in response to elevated extracellular copper levels where it functions in the efflux of copper from cells. Defects in ATP7A are the cause of Menkes syndrome (MD) and occipital horn syndrome (OHS). Six alternatively spliced isoforms have been described. The longest isoform has 6 transmembrane regions. Isoform 3, lacking all 6 transmembrane regions and 5 heavy-metal-associated (HMA) domains, is probably cytosolic. Isoform 6 lacks all transmembrane regions and 5 heavy-metal-associated (HMA) domains, but has a putative nuclear localization signal attached at the N-terminus.

Protein type: Transporter, ion channel; Hydrolase; Vesicle; Cell development/differentiation; EC 3.6.3.54; Membrane protein, multi-pass; Membrane protein, integral; Chaperone; Apoptosis; Transporter

Chromosomal Location of Human Ortholog: Xq21.1

Cellular Component: Golgi apparatus; neuron projection; brush border membrane; basolateral plasma membrane; endoplasmic reticulum; integral to membrane; trans-Golgi network; cytosol; secretory granule; trans-Golgi network transport vesicle; membrane; cell soma; perinuclear region of cytoplasm; late endosome; plasma membrane

Molecular Function: copper ion transmembrane transporter activity; protein binding; copper ion binding; copper-exporting ATPase activity; superoxide dismutase copper chaperone activity; ATP binding; copper-dependent protein binding

Biological Process: skin development; positive regulation of catalytic activity; extracellular matrix organization and biogenesis; collagen fibril organization; catecholamine metabolic process; dopamine metabolic process; norepinephrine biosynthetic process; tryptophan metabolic process; plasma membrane copper ion transport; negative regulation of neuron apoptosis; central nervous system neuron development; positive regulation of oxidoreductase activity; mitochondrion organization and biogenesis; release of cytochrome c from mitochondria; response to iron(III) ion; dendrite morphogenesis; detoxification of copper ion; ATP metabolic process; elastic fiber assembly; response to reactive oxygen species; response to zinc ion; regulation of gene expression; regulation of oxidative phosphorylation; cerebellar Purkinje cell differentiation; copper ion import; alveolus development; lactation; removal of superoxide radicals; locomotory behavior; pyramidal neuron development; norepinephrine metabolic process; peptidyl-lysine modification; epinephrine metabolic process; transmembrane transport; serotonin metabolic process; blood vessel development; negative regulation of metalloenzyme activity; hair follicle morphogenesis; cellular copper ion homeostasis; T-helper cell differentiation; in utero embryonic development; tyrosine metabolic process; positive regulation of metalloenzyme activity; copper ion transport; elastin biosynthetic process; pigmentation; cartilage development; blood vessel remodeling; copper ion export; neurite morphogenesis

Disease: Occipital Horn Syndrome; Menkes Disease; Spinal Muscular Atrophy, Distal, X-linked 3
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Research Articles on ATP7A

1. This is the first time the activation of pseudo-exons is demonstrated in the ATP7A gene, and it demonstrates the usefulness of RNA analysis, in terms of revealing disease-causing mutations in noncoding regions.

Precautions

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

Disclaimer

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.

Product Note

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual, it is intended to serve as an example only. Please refer to the instructions For Use provided with the assay kit for precise details.

Product PubMed Publications

Products associated with anti-ATP7A antibody

 Reference Product  PubMed Publications
 ATOX1 antibody  >59 publications with ATP7A and ATOX1
 COMMD1 antibody  >9 publications with ATP7A and COMMD1
 SOD3 antibody  >5 publications with ATP7A and SOD3
 ATRX antibody  >2 publications with ATP7A and ATRX
 PDZD11 antibody  >1 publications with ATP7A and PDZD11

Pathways associated with anti-ATP7A antibody

 Products by Pathway  Pathway Diagram
 Cellular Responses To Stress Pathway antibodies  Cellular Responses To Stress Pathway Diagram
 Detoxification Of Reactive Oxygen Species Pathway antibodies  Detoxification Of Reactive Oxygen Species Pathway Diagram
 Ion Channel Transport Pathway antibodies  Ion Channel Transport Pathway Diagram
 Ion Transport By P-type ATPases Pathway antibodies  Ion Transport By P-type ATPases Pathway Diagram
 Mineral Absorption Pathway antibodies  Mineral Absorption Pathway Diagram
 Mineral Absorption Pathway antibodies  Mineral Absorption Pathway Diagram
 Transmembrane Transport Of Small Molecules Pathway antibodies  Transmembrane Transport Of Small Molecules Pathway Diagram

Diseases associated with anti-ATP7A antibody

 Disease Name  Pubmed Publications
 Nervous System Diseases Antibodies  >292 publications with ATP7A and Nervous System Diseases
 Brain Diseases Antibodies  >281 publications with ATP7A and Brain Diseases
 Menkes Kinky Hair Syndrome Antibodies  >229 publications with ATP7A and Menkes Kinky Hair Syndrome
 Liver Diseases Antibodies  >89 publications with ATP7A and Liver Diseases
 Disease Models, Animal Antibodies  >67 publications with ATP7A and Disease Models, Animal
 Occipital horn syndrome Antibodies  >39 publications with ATP7A and Occipital horn syndrome
 Congenital Abnormalities Antibodies  >25 publications with ATP7A and Congenital Abnormalities
 Gastrointestinal Diseases Antibodies  >9 publications with ATP7A and Gastrointestinal Diseases
 Adenocarcinoma Antibodies  >9 publications with ATP7A and Adenocarcinoma
 Neoplasms, Experimental Antibodies  >9 publications with ATP7A and Neoplasms, Experimental

Organs/Tissues associated with anti-ATP7A antibody

 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >114 publications with ATP7A and Brain
 Liver Antibodies  >92 publications with ATP7A and Liver
 Blood Antibodies  >69 publications with ATP7A and Blood
 Kidney Antibodies  >30 publications with ATP7A and Kidney
 Connective Tissue Antibodies  >29 publications with ATP7A and Connective Tissue
 Vascular Antibodies  >27 publications with ATP7A and Vascular
 Skin Antibodies  >20 publications with ATP7A and Skin
 Muscle Antibodies  >18 publications with ATP7A and Muscle
 Lung Antibodies  >17 publications with ATP7A and Lung
 Heart Antibodies  >15 publications with ATP7A and Heart
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