Recombinant Human ATP7B Protein, Myc/ddk-tagged

• Cat.No.: ATP7B-468H
• Product Overview: Recombinant protein of human ATPase, Cu++ transporting, beta polypeptide (ATP7B), transcript variant 1 with a C-Myc/DDK tag was expressed in HEK293T.

Specification

• Description: This gene is a member of the P-type cation transport ATPase family and encodes a protein with several membrane-spanning domains, an ATPase consensus sequence, a hinge domain, a phosphorylation site, and at least 2 putative copper-binding sites. This protein is a monomer, and functions as a copper-transporting ATPase which exports copper out of the cells, such as the efflux of hepatic copper into the bile. Alternate transcriptional splice variants, encoding different isoforms with distinct cellular localizations, have been characterized. Mutations in this gene have been associated with Wilson disease which is characterized by copper accumulation.
• Source: HEK293T
• Species: Human
• Tag: Myc/DDK
• Molecular Mass: 157.1 kDa
• Purity: > 80% as determined by SDS-PAGE and Coomassie blue staining
• Concentration: >50 μg/mL as determined by microplate BCA method
• Storage Buffer: 25 mM Tris.HCl, pH 7.3, 100 mM glycine, 10% glycerol

Gene Information

• Gene Name: ATP7B ATPase copper transporting beta [ Homo sapiens (human) ]
• Official Symbol: ATP7B
• Synonyms: ATP7B; ATPase copper transporting beta; WD; PWD; WC1; WND; copper-transporting ATPase 2; ATPase, Cu(2+)- transporting, beta polypeptide; ATPase, Cu++ transporting, beta polypeptide; Wilson disease-associated protein; copper pump 2; copper-transporting protein ATP7B; EC 7.2.2.8
• Gene ID: 540
• mRNA Refseq: NM_000053
• Protein Refseq: NP_000044
• MIM: 606882
• UniProt ID: P35670

Reviews

01/15/2021

Impressed with the product quality.

05/09/2020

The product exceeded expectations.

09/28/2018

This product consistently delivers accurate results in our experiments.

Q&As

How does the ATP7B protein contribute to copper transport within cells, and what cellular compartments are involved in this process?

ATP7B facilitates copper transport from the cytoplasm to the Golgi apparatus, where copper is incorporated into proteins. It also mediates copper export from cells when copper levels are elevated, preventing toxicity.

How does ATP7B interact with other proteins involved in copper metabolism, and what are the implications of disruptions in these interactions?

ATP7B interacts with various proteins in the copper transport pathway. Disruptions in these interactions can lead to mislocalization of ATP7B and compromise its ability to regulate copper levels properly.

How does ATP7B-mediated copper transport impact the activity of copper-dependent enzymes, and what are the broader implications for cellular function?

ATP7B ensures the proper supply of copper to enzymes involved in various cellular processes, including energy production and antioxidant defense. Dysregulation can disrupt these processes, affecting cell function.

What is the role of ATP7B in copper excretion at the systemic level, and how does this relate to copper metabolism in the body?

ATP7B is crucial for copper excretion into bile, a process that contributes to systemic copper balance. Dysfunctional ATP7B results in impaired copper excretion, leading to the accumulation of copper in tissues.

Can you explain the consequences of mutations in the ATP7B gene, particularly in relation to Wilson's disease?

Mutations in the ATP7B gene lead to impaired copper transport, causing copper accumulation in tissues. This is associated with Wilson's disease, a disorder characterized by copper toxicity, especially in the liver and brain.