Recombinant Human ATP7A protein is a Human Fragment protein, in the 1406 to 1500 aa range, expressed in Wheat germ and suitable for SDS-PAGE, ELISA, WB.
Images
Key facts
- Expression system
- Wheat germ
- Tags
- Tag free
- Applications
- SDS-PAGE, ELISA, WB
- Biologically active
- No
Amino acid sequence
F L K L Y R K P T Y E S Y E L P A R S Q I G Q K S P S E I S V H V G I D D T S R N S P K L G L L D R I V N Y S R A S I N S L L S D K R S L N S V V T S E P D K H S L L V G D F R E D D D T A L
Reactivity data
| Application | Reactivity | Dilution info | Notes |
|---|---|---|---|
Application SDS-PAGE | Reactivity Reacts | Dilution info - | Notes - |
Application ELISA | Reactivity Reacts | Dilution info - | Notes - |
Application WB | Reactivity Reacts | Dilution info - | Notes (for recombinant protein). |
Target data
Function
ATP-driven copper (Cu(+)) ion pump that plays an important role in intracellular copper ion homeostasis (PubMed:10419525, PubMed:11092760, PubMed:28389643). Within a catalytic cycle, acquires Cu(+) ion from donor protein on the cytoplasmic side of the membrane and delivers it to acceptor protein on the lumenal side. The transfer of Cu(+) ion across the membrane is coupled to ATP hydrolysis and is associated with a transient phosphorylation that shifts the pump conformation from inward-facing to outward-facing state (PubMed:10419525, PubMed:19453293, PubMed:19917612, PubMed:28389643, PubMed:31283225). Under physiological conditions, at low cytosolic copper concentration, it is localized at the trans-Golgi network (TGN) where it transfers Cu(+) ions to cuproenzymes of the secretory pathway (PubMed:11092760, PubMed:28389643). Upon elevated cytosolic copper concentrations, it relocalizes to the plasma membrane where it is responsible for the export of excess Cu(+) ions (PubMed:10419525, PubMed:28389643). May play a dual role in neuron function and survival by regulating cooper efflux and neuronal transmission at the synapse as well as by supplying Cu(+) ions to enzymes such as PAM, TYR and SOD3 (By similarity) (PubMed:28389643). In the melanosomes of pigmented cells, provides copper cofactor to TYR to form an active TYR holoenzyme for melanin biosynthesis (By similarity).
Alternative names
MC1, MNK, ATP7A, Copper-transporting ATPase 1, Copper pump 1, Menkes disease-associated protein
Recommended products
Recombinant Human ATP7A protein is a Human Fragment protein, in the 1406 to 1500 aa range, expressed in Wheat germ and suitable for SDS-PAGE, ELISA, WB.
Key facts
- Expression system
- Wheat germ
- Tags
- Tag free
- Applications
- SDS-PAGE, ELISA, WB
- Biologically active
- No
- Accession
- Q04656-1
- Animal free
- No
- Species
- Human
- Concentration
- Storage buffer
pH: 8
Constituents: 0.79% Tris HCl, 0.3% Glutathione
Sequence info
Amino acid sequence
- F L K L Y R K P T Y E S Y E L P A R S Q I G Q K S P S E I S V H V G I D D T S R N S P K L G L L D R I V N Y S R A S I N S L L S D K R S L N S V V T S E P D K H S L L V G D F R E D D D T A L
- Accession
- Q04656
- Protein length
- Fragment
- Predicted molecular weight
- 36.08 kDa
- Amino acids
- 1406 to 1500
- Nature
- Recombinant
Specifications
- Form
- Liquid
General info
- Function
ATP-driven copper (Cu(+)) ion pump that plays an important role in intracellular copper ion homeostasis (PubMed:10419525, PubMed:11092760, PubMed:28389643). Within a catalytic cycle, acquires Cu(+) ion from donor protein on the cytoplasmic side of the membrane and delivers it to acceptor protein on the lumenal side. The transfer of Cu(+) ion across the membrane is coupled to ATP hydrolysis and is associated with a transient phosphorylation that shifts the pump conformation from inward-facing to outward-facing state (PubMed:10419525, PubMed:19453293, PubMed:19917612, PubMed:28389643, PubMed:31283225). Under physiological conditions, at low cytosolic copper concentration, it is localized at the trans-Golgi network (TGN) where it transfers Cu(+) ions to cuproenzymes of the secretory pathway (PubMed:11092760, PubMed:28389643). Upon elevated cytosolic copper concentrations, it relocalizes to the plasma membrane where it is responsible for the export of excess Cu(+) ions (PubMed:10419525, PubMed:28389643). May play a dual role in neuron function and survival by regulating cooper efflux and neuronal transmission at the synapse as well as by supplying Cu(+) ions to enzymes such as PAM, TYR and SOD3 (By similarity) (PubMed:28389643). In the melanosomes of pigmented cells, provides copper cofactor to TYR to form an active TYR holoenzyme for melanin biosynthesis (By similarity).
- Sequence similarities
Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily.
- Subcellular localisation
- Early endosome membrane
Storage
- Shipped at conditions
- Dry Ice
- Appropriate short-term storage conditions
- -80°C
- Appropriate long-term storage conditions
- -80°C
- Aliquoting information
- Upon delivery aliquot
- Storage information
- Avoid freeze / thaw cycle
Supplementary info
- This supplementary information is collated from multiple sources and compiled automatically.
- Activity summary
ATP7A also known as Menkes protein is a copper-transporting ATPase. It has a molecular weight of approximately 162 kDa. ATP7A helps transport copper within cells by being primarily located in the trans-Golgi network and is also found in the plasma membrane. This protein has significant expression in several tissues especially in the nervous system intestines and connective tissue. ATP7A plays a role in distributing copper to copper-dependent enzymes critical for various cellular processes.
- Biological function summary
ATP7A is essential in maintaining copper homeostasis across multiple systems. It is involved in supplying copper to enzymes like lysyl oxidase which is important for connective tissue development. ATP7A does not function as a part of a larger complex but it coordinates closely with cellular mechanisms ensuring appropriate copper levels. Its movement between golgi apparatus and the cell membrane allows for effective intracellular copper management.
- Pathways
ATP7A influences copper metabolic pathways and is specifically important in the absorption and distribution of copper. It works with another transporter protein ATP7B to maintain copper balance by exporting excess copper into the bile and plasma. Both proteins are critical in copper regulation and show complementary functions in preventing copper toxicity.
- Associated diseases and disorders
ATP7A is closely associated with Menkes disease a disorder characterized by defective copper absorption and distribution leading to developmental delays and connective tissue abnormalities. Another related condition is Occipital Horn Syndrome which involves mutations in ATP7A. These diseases highlight ATP7A's key role in copper transport and highlight its interaction with ATP7B in copper homeostasis directly linking these proteins to the pathology of the disorders.
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1 product image
SDS-PAGE - Recombinant Human ATP7A protein (ab114343)
ab114343 analysed on a 12.5% SDS-PAGE gel stained with Coomassie Blue.
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