Human Copper-transporting ATPase 1 ELISA Kit | ATP7A elisa kit

Human Copper-transporting ATPase 1 ELISA Kit

Cat. Num. AAA764629

• Gene Names: ATP7A; MK; MNK; DSMAX; SMAX3
• Reactivity: Human
• Synonyms: Copper-transporting ATPase 1; Human Copper-transporting ATPase 1 ELISA Kit; ATP7A/Copper-transporting ATPase 1/Copper pump 1/Menkes disease-associated protein/MC1/MNK/DSMAX/SMAX3/MK; ATP7A elisa kit

• Reactivity: Human
• Sequence Length: 1500

• Samples: Serum, plasma and other biological fluids.
• Assay Type: Sandwich ELISA, Double Antibody
• Detection Range: 0.156-10ng/ml
• Sensitivity: 0.094ng/ml
• Preparation and Storage: Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Typical Testing Data

Typical Standard Curve

Related Product Information for ATP7A elisa kit

Principle of the Assay||This kit was based on sandwich enzyme-linked immune-sorbent assay technology. Anti-Human ATP7A antibody was pre-coated onto 96-well plates. And the biotin conjugated anti-Human ATP7A antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and wash with wash buffer. HRP-Streptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the Human ATP7A amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration of Human ATP7A can be calculated.

NCBI and Uniprot Product Information

• NCBI GI #: 532691750
• NCBI GeneID: 538
• NCBI Accession #: NP_000043.4
• NCBI GenBank Nucleotide #: NM_000052.6
• Molecular Weight: 11,522 Da
• NCBI Official Full Name: copper-transporting ATPase 1 isoform 1
• NCBI Official Synonym Full Names: ATPase copper transporting alpha
• NCBI Official Symbol: ATP7A
• NCBI Official Synonym Symbols: MK; MNK; DSMAX; SMAX3
• NCBI Protein Information: copper-transporting ATPase 1
• UniProt Protein Name: Copper-transporting ATPase 1
• Protein Family: Copper-transporting ATPase
• UniProt Gene Name: ATP7A
• UniProt Synonym Gene Names: MC1; MNK
• UniProt Entry Name: ATP7A_HUMAN

NCBI Description

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 Description

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

Chromosomal Location of Human Ortholog: Xq21.1

Cellular Component: basolateral plasma membrane; cell soma; Golgi apparatus; integral to plasma membrane; late endosome; membrane; neuron projection; perinuclear region of cytoplasm; plasma membrane; trans-Golgi network; trans-Golgi network transport vesicle

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

Biological Process: alveolus development; blood vessel development; blood vessel remodeling; cartilage development; catecholamine metabolic process; cellular copper ion homeostasis; central nervous system neuron development; cerebellar Purkinje cell differentiation; collagen fibril organization; copper ion export; copper ion import; copper ion transport; detoxification of copper ion; dopamine metabolic process; elastic fiber assembly; elastin biosynthetic process; epinephrine metabolic process; extracellular matrix organization and biogenesis; hair follicle morphogenesis; locomotory behavior; mitochondrion organization and biogenesis; negative regulation of catalytic activity; neurite morphogenesis; norepinephrine metabolic process; peptidyl-lysine modification; pigmentation; positive regulation of catalytic activity; positive regulation of oxidoreductase activity; pyramidal neuron development; regulation of oxidative phosphorylation; removal of superoxide radicals; serotonin metabolic process; skin development; T-helper cell differentiation; tryptophan metabolic process

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