Anti-ATP7A Antibody Picoband®

SKU: A01085-1

Size: 100 μg

Storage: 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.

Form: Lyophilized

Reactivity: Human

Applications: WB,Flow Cytometry

Application Details: Western blot, 0.25-0.5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human

Gene Name: ATP7A

Specificity:

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.

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

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

Purification: Immunogen affinity purified.

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

Reference: 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.

Uniprot ID: Q04656

Host: Rabbit

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

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 163 kDa

Calculated Molecular Weight: 163 kDa

Gene ID: 538

Protein Name: Copper-transporting ATPase 1

Gene Full Name: ATPase copper transporting alpha

Synonyms: Copper pump 1; DSMAX; MK; MNK; SMAX3; ATP7A; MC1