ATP7A, Polyclonal Antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

This antibody is will react to isoforms 1-5.

Affinity Purified

Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

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.

This is a rabbit polyclonal antibody against ATP7A. It was validated on Western Blot using a cell lysate as a positive control.

Target Description: The ATP7A gene encodes the Menkes copper-translocating P-type ATPase, a ubiquitous protein that regulates the absorption of copper in the gastrointestinal tract. Inside cells, this protein has a dual function: it delivers copper to cuproenzymes in the Gol

Polyclonal; Membrane Protein; Drugs and Drug Metabolism; E3 & Ubiquitin; Phosphorylation; Cell Differentiation;

Western Blot (WB)

Lanes:
Lane1: 40 ug mouse endothelial firboblast lysate
Lane2: 40 ug human HUVEC lysate
Primary Antibody Dilution:
1:500
Secondary Antibody:
Anti-rabbit HRP
Secondary Antibody Dilution:
1:2000
Gene Name:
ATP7A
Submitted by:
Anonymous

WB Suggested Anti-ATP7A Antibody Titration: 0.2-1 ug/ml
ELISA Titer: 1:312500
Positive Control: HT1080 cell lysateATP7A is supported by BioGPS gene expression data to be expressed in HT1080

163kDa

ATPase copper transporting alpha

copper-transporting ATPase 1

Copper-transporting ATPase 1

MC1; MNK  [Similar Products]

ATP7A_HUMAN

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]

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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