Product Name
ATP7A, siRNA
Full Product Name
ATP7A siRNA (Human)
Product Synonym Names
MC1; MNK; Copper-transporting ATPase 1; Copper pump 1; Menkes disease-associated protein
Product Gene Name
ATP7A sirna
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q04656
Specificity
ATP7A siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
Purity/Purification
> 97%
Form/Format
Lyophilized powder
Quality Control
Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
Directions for Use
We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
Components
We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human ATP7A gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
Preparation and Storage
Shipped at 4 degree C. Store at -20 degree C for one year.
Negative Control
siRNA Negative Control (Catalog# MBS8241404) is a non-targeting 21 nt siRNA recommended as a negative control for experiments using targeted siRNA transfection.
Other Notes
Small volumes of ATP7A sirna 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.
Related Product Information for
ATP7A sirna
siRNA to inhibit ATP7A expression using RNA interference
Applications Tested/Suitable for ATP7A sirna
RNA Interference (RNAi)
NCBI/Uniprot data below describe general gene information for ATP7A. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000043.4
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NCBI GenBank Nucleotide #
NM_000052.6
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UniProt Primary Accession #
Q04656
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UniProt Secondary Accession #
O00227; O00745; Q9BYY8; B1AT72[Other Products]
UniProt Related Accession #
Q04656[Other Products]
Molecular Weight
11,522 Da
NCBI Official Full Name
copper-transporting ATPase 1 isoform 1
NCBI Official Synonym Full Names
ATPase, Cu++ transporting, alpha polypeptide
NCBI Official Symbol
ATP7A [Similar Products]
NCBI Official Synonym Symbols
MK; MNK; DSMAX; SMAX3
[Similar Products]
NCBI Protein Information
copper-transporting ATPase 1
UniProt Protein Name
Copper-transporting ATPase 1
UniProt Synonym Protein Names
Copper pump 1; Menkes disease-associated protein
UniProt Gene Name
ATP7A [Similar Products]
UniProt Synonym Gene Names
MC1; MNK [Similar Products]
UniProt Entry Name
ATP7A_HUMAN
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]
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: Vesicle; Chaperone; Apoptosis; Transporter; Transporter, ion channel; Membrane protein, integral; Membrane protein, multi-pass; Cell development/differentiation; Hydrolase; EC 3.6.3.54
Chromosomal Location of Human Ortholog: Xq21.1
Cellular Component: Golgi apparatus; neuron projection; endoplasmic reticulum; brush border membrane; basolateral plasma membrane; integral to membrane; trans-Golgi network; cytosol; secretory granule; trans-Golgi network transport vesicle; cell soma; membrane; 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; copper-dependent protein binding; ATP 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; regulation of gene expression; response to zinc ion; 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; cellular copper ion homeostasis; hair follicle morphogenesis; 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
Research Articles on ATP7A
1. Depletion of CCC complex components leads to lack of copper-dependent movement of the copper transporter ATP7A from endosomes, resulting in intracellular copper accumulation and modest alterations in copper homeostasis in humans with CCDC22 mutations.
Precautions
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