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  4. Recombinant Mouse Copper-transporting ATPase 1 (Atp7a), partial

Recombinant Mouse Copper-transporting ATPase 1 (Atp7a), partial

  • 中文名称：
    Recombinant Mouse Copper-transporting ATPase 1(Atp7a) ,partial
  • 货号：
    CSB-YP727071MO
  • 规格：
  • 来源：
    Yeast
  • 其他：
  • 中文名称：
    Recombinant Mouse Copper-transporting ATPase 1(Atp7a) ,partial
  • 货号：
    CSB-EP727071MO
  • 规格：
  • 来源：
    E.coli
  • 其他：
  • 中文名称：
    Recombinant Mouse Copper-transporting ATPase 1(Atp7a) ,partial
  • 货号：
    CSB-EP727071MO-B
  • 规格：
  • 来源：
    E.coli
  • 共轭：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 中文名称：
    Recombinant Mouse Copper-transporting ATPase 1(Atp7a) ,partial
  • 货号：
    CSB-BP727071MO
  • 规格：
  • 来源：
    Baculovirus
  • 其他：
  • 中文名称：
    Recombinant Mouse Copper-transporting ATPase 1(Atp7a) ,partial
  • 货号：
    CSB-MP727071MO
  • 规格：
  • 来源：
    Mammalian cell
  • 其他：

产品详情

  • 纯度：
    >85% (SDS-PAGE)
  • 基因名：
    Atp7a
  • Uniprot No.：
    Q64430
  • 别名：
    Atp7a; MnkCopper-transporting ATPase 1; EC 7.2.2.8; Copper pump 1; Menkes disease-associated protein homolog
  • 种属：
    Mus musculus (Mouse)
  • 蛋白长度：
    Partial
  • 蛋白标签：
    Tag type will be determined during the manufacturing process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 产品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 复溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

产品评价

靶点详情

  • 功能：
    ATP-driven copper (Cu(+)) ion pump that plays an important role in intracellular copper ion homeostasis. 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. 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. Upon elevated cytosolic copper concentrations, it relocalizes to the plasma membrane where it is responsible for the export of excess Cu(+) ions. 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. In the melanosomes of pigmented cells, provides copper cofactor to TYR to form an active TYR holoenzyme for melanin biosynthesis.
  • 基因功能参考文献：
    1. Copper therapy reduces intravascular hemolysis and derepresses ferroportin in mice with mosaic mutation (Atp7a(mo-ms)): An implication for copper-mediated regulation of the Slc40a1 gene expression. PMID: 28219768
    2. we demonstrated that the restoration/preservation of autophagic-lysosomal degradation in senescent MEFs following rapamycin treatment correlated with attenuation of copper accumulation in these cells despite a further decrease in Atp7a levels. This study for the first time establishes a link between Atp7a and the autophagic-lysosomal pathway, and a requirement for both to effect efficient copper export PMID: 29579719
    3. Copper homeostasis in the testes is closely controlled by copper-transporting ATPases ATP7A and ATP7B proteins. PMID: 28820536
    4. Atp7a(T985I/Y) mice have reduced Atp7a protein levels and recapitulate the defective trafficking and altered post-translational regulatory mechanisms observed in the human ATP7A(T994I) patient fibroblasts PMID: 27293072
    5. Thus, ATP7A activity protects mitochondria from excessive copper entry, which is deleterious to redox buffers. Mitochondrial redox misbalance could significantly contribute to pathologies associated with ATP7A inactivation in tissues with paradoxical accumulation of copper PMID: 27226607
    6. CTR1, ATP7A, and lysyl oxidase were upregulated in the lung tissues and pulmonary arteries of mice with hypoxia-induced pulmonary hypertension and pulmonary arterial smooth muscle cells. PMID: 24614111
    7. study characterized the Mottled-dappled deletion in C3H101H carrier females PMID: 25456742
    8. Results from experiments in mouse model of Menkes disease harboring ATP5a mutation, show increase expression of SOD1 and induction of HO-1 causing iron metabolism disruption and hemolysis due to copper deficiency. PMID: 25247420
    9. The MPhi ATP7A selectively regulates LPS-induced inflammatory mediators, in part, via modulation of cellular copper availability, whereas neocuproine generally inhibits the production of inflammatory mediators. PMID: 23604539
    10. a decrease in ATP7A protein expression contributes to impaired SOD3 activity, resulting in O2(*-) overproduction and endothelial dysfunction in blood vessels of type 1 diabetes mellitus. PMID: 23884884
    11. ATP7A mutations leading to Menkes disease and occipital horn syndrome in human and animal models [Review] PMID: 23281160
    12. These studies demonstrate the essential role of the Atp7a gene in mouse embryonic development and establish a powerful model for understanding the tissue-specific roles of ATP7A in copper metabolism and disease. PMID: 22900086
    13. ATP7A-mediated copper homeostasis is important for the formation of pathogenic proteinase-resistant prion protein PMID: 22869751
    14. In the present study we identified a G to C nucleotide exchange in exon 15 of the Atp7a gene in mosaic mutants, which resulted in an arginine to proline substitution in the highly conserved 6th transmembrane domain of the ATP7A protein. PMID: 22089129
    15. macrophage ATP7A is localized in the trans-Golgi apparatus, regulates intracellular copper levels, and mediates macrophage responses to a dermal wound PMID: 21336677
    16. Mutations in ATP7A is associated with lethal infantile neurodegenerative disorder of copper metabolism PMID: 21878905
    17. Clusterin and COMMD1 independently regulate degradation of the mammalian copper ATPases ATP7A and ATP7B. PMID: 22130675
    18. Study analysed the expression of the Atp7a gene in the brain, kidney and liver of newborn and suckling mice; in 2 week-old mutants, a significant decrease was observed only in the kidney. PMID: 20831904
    19. sperm quality of mosaic mutants, measured by sperm head morphology, vitality, tail membrane integrity, motility, and maturation, is strongly affected. Copper deficiency negatively influences antioxidative defense PMID: 20849226
    20. Atp7b(-/-) mice demonstrated decreased serum iron parameters and hemoglobin levels most likely related to a low serum ceruloplasmin oxidase activity and not due to total body iron deficiency. PMID: 20594231
    21. Atp7A plays an important role in copper-dependent vascular smooth muscle cell migration and may contribute to neointimal formation after experimental vascular injury. PMID: 20671235
    22. a novel mechanism linking ATP7A to cPLA(2)alpha and LDL oxidation PMID: 19965596
    23. The expression of the Atp7a gene in the liver in mice is age-dependent and decreases during postnatal development. PMID: 20084666
    24. Both Atp7a and Atp7b are expressed in glomeruli PMID: 12372948
    25. The mosaic mutation is caused by changes in the protein responsible for ATP-dependent copper transport across cell membranes. Copper deficiency causes pathological changes to the median eminence and impairs somatostatin expression. PMID: 15159688
    26. The Mobr allele causes a severe ( approximately 60%) depletion of spinal cord copper levels; however, despite the burden of double genetic lesions, it lengthens the lives of SOD1(G86R) transgenic mice by 9%. PMID: 15356208
    27. Distinct functions of ATP7A and ATP7B in the cerebellum and illustrate a tight link between copper homeostasis in Purkinje neuerons and Bergmann glia. PMID: 15634671
    28. These data reveal a critical role for Menkes ATPase in the availability of an NMDA receptor-dependent pool of copper in hippocampal neurons and demonstrate a mechanism linking copper homeostasis and neuronal activation within the CNS. PMID: 15634787
    29. The most severe model of human Menkes disease in mottled mice established to date is caused by a defect in the Atp7a gene and is useful for understanding the gene function in Menkes disease. PMID: 16338116
    30. Vascular MNK plays an essential role in full activity of SOD3 through transporting copper to SOD3 in the TGN, thereby regulating superoxide levels in the vasculature. PMID: 16371425
    31. These data reveal a unique connection between copper homeostasis and NMDA receptor activity that is of broad relevance to the processes of synaptic plasticity and excitotoxic cell death. PMID: 17003121
    32. Morphological and functional alterations of the testes are mainly the result of copper deficiency in mosaic mice and excess estrogens could decrease the survival rate of mosaic males PMID: 17045330
    33. This study demonstrates a role for ATP7A and/or copper in axon outgrowth and synaptogenesis. PMID: 17215139
    34. Menkes disease phenotypic diversity in mottled mice is associated with defects in localisation and trafficking of the ATP7A protein PMID: 17483305
    35. examined ATP7A expression in the CNS of MObry/y mice to search fro compensatory alterations that may occur in response to copper deficiency. PMID: 17588765
    36. Both Cu-ATPases (ATP7A and ATP7b) regulate renal copper, with ATP7A playing a major role in exporting copper via basolateral membranes and protecting renal tissue against copper overload. PMID: 17928409
    37. review of quantitative detection of copper-transporting ATPases in tissues and distinct biochemical characteristics and dissimilar trafficking properties of ATP7A and ATP7B in cells, in which they are co-expressed, indicating distinct specific functions PMID: 18000748
    38. Copper is supplied to melanosomes by ATP7A, a cohort of which localizes to melanosomes in a biogenesis of lysosome-related organelles complex-1 (BLOC-1)-dependent manner PMID: 18650808
    39. Copper transporter Menkes ATPase (MNK) plays a role in modulating Ang II-induced hypertension and endothelial function by regulating SOD3 activity and vascular superoxide anion production. PMID: 18768397
    40. The cDNA of Atp7a was sequenced from 9 unrelated mutants and 7 unrelated control mice. It was found that a CAG insertion at the end of the 4th exon exists in the mutants but not in control cDNA. PMID: 19058563
    41. copper-transporting ATPases, CopA and ATP7A, in both bacteria and macrophage are unique determinants of bacteria survival and identify an unexpected role for copper at the host-pathogen interface PMID: 19808669

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  • 相关疾病：
    Defects in Atp7a are associated with mottled, an X-linked recessive condition characterized by mottled pigmentation of the coat, defects in connective tissue and neonatal or fetal death. It is due to a defect in absorption and transport of copper. The mottled mutants exhibit a diversity of phenotypes. Two of these mutants are called brindled and blotchy and their phenotypes resemble classical Menkes disease (MD) and occipital horn syndrome (OHS) in humans, respectively. Other mutants are called dappled, mosaic, tortoiseshell, pewter, etc.
  • 亚细胞定位：
    Golgi apparatus, trans-Golgi network membrane; Multi-pass membrane protein. Cell membrane; Multi-pass membrane protein. Melanosome membrane; Multi-pass membrane protein. Early endosome membrane; Multi-pass membrane protein. Cell projection, axon. Cell projection, dendrite. Cell junction, synapse, postsynaptic density.
  • 蛋白家族：
    Cation transport ATPase (P-type) (TC 3.A.3) family, Type IB subfamily
  • 组织特异性：
    Widely expressed. Highly expressed in pituitary endocrine cells. Expressed in melanocytes (at protein level). Expressed in motor neuron (at protein level). Expressed in hippocampal neuron (at protein level). In the kidney, it is detected in the proximal a
  • 数据库链接：

    KEGG: mmu:11977

    STRING: 10090.ENSMUSP00000058840

    UniGene: Mm.254297

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