Abcam, ab131400, Anti-ATP7A antibody [L60/4]

Size: 100µg
Mouse Monoclonal ATP7A antibody. Suitable for WB, ICC/IF and reacts with Rat, Mouse, Human samples. Cited in 4 publications. Immunogen corresponding to Synthetic Peptide within Human ATP7A aa 1-100.
Key facts
Host species:Mouse,
Clonality:Monoclonal,
Clone number:L60/4,
Isotype:IgG2b,
Carrier free:No,
Reacts with:Mouse, Human, Rat,
Applications:WB, ICC/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Synthetic Peptide within Human ATP7A aa 1-100. The exact immunogen used to generate this antibody is proprietary information.Q04656

Product details:
The clone number has been updated from S60-4 to L60/4, both clone numbers name the same antibody clone.

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Protein G, Storage buffer-Preservative: 0.09% Sodium azideConstituents: PBS, 50% Glycerol (glycerin, glycerine), Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
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.
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.