Anti-ATP7A Antibody Picoband®, iFluor647 Conjugate

Boster Bio Anti-ATP7A Antibody Picoband® catalog # A01085-1. Tested in WB, Flow Cytometry applications. This antibody reacts with Human. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Product Specifications

Background

ATP7A, also known as Menkes' protein (MNK), is a copper-transporting P-type ATPase which uses the energy arising from ATP hydrolysis to transport Cu(I) across cell membranes. 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.

Synonyms

ATP7A; MC1; MNK; Copper-transporting ATPase 1; EC 7.2.2.8; Copper pump 1; Menkes disease-associated protein

Gene Name

ATPase copper transporting alpha

Gene ID

538

UniProt

Q04656

Host

Rabbit

Reactivity

Human

Cross Reactivity

No cross-reactivity with other proteins

Immunogen

A synthetic peptide corresponding to a sequence at the C-terminus of human ATP7A, which shares 79.2% amino acid (aa) sequence identity with both mouse and rat ATP7A.

Clonality

Polyclonal

Applications

WB,Flow Cytometry

Purification

Immunogen affinity purified.

Concentration

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Form

Lyophilized

Reconstitution

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Components

Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

References & Citations

1. Cecchi, C., Biasotto, M., Tosi, M., Avner, P. The mottled mouse as a model for human Menkes disease: identification of mutations in the Atp7a gene. Hum. Molec. Genet. 6: 425-433, 1997. Note: Erratum: Hum. Molec. Genet. 6: 829 only, 1997. 2. Chelly, J., Tumer, Z., Tonnesen, T., Petterson, A., Ishikawa-Brush, Y., Tommerup, N., Horn, N., Monaco, A. P. Isolation of a candidate gene for Menkes disease that encodes a potential heavy metal binding protein. Nature Genet. 3: 14-19, 1993. 3. Christodoulou, J., Danks, D. M., Sarkar, B., Baerlocher, K. E., Casey, R., Horn, N., Tumer, Z., Clarke, J. T. R. Early treatment of Menkes disease with parenteral cooper (sic)-histidine: long-term follow-up of four treated patients. Am. J. Med. Genet. 76: 154-164, 1998.

Storage Conditions

At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.