ATP7B (ATPase copper transporting beta) is a transmembrane ATPase protein responsible for regulating the transport and metabolism of copper ions within cells. Its structure includes multiple functional domains, including several transport domains and domains for catalyzing ATP hydrolysis. These domains work in synergy to enable ATP7B to transport copper ions across the cell membrane. ATP7B is primarily expressed in subcellular compartments of liver cells with high copper content, such as the copper-rich Golgi apparatus and cytoplasmic lysosomes. It is involved in the intracellular transport and distribution of copper ions, shuttling copper from the cytoplasm to the Golgi apparatus or releasing it from the Golgi apparatus to cytoplasmic lysosomes. The function of ATP7B is critical for maintaining copper balance within the body and preventing copper accumulation to toxic levels. Copper is an essential element for life processes but can be toxic to cells and tissues at high concentrations. Therefore, aberrant function or mutations of ATP7B can lead to Wilson's disease, a hereditary copper metabolism disorder. Research on ATP7B is crucial for understanding the regulatory mechanisms of copper metabolism and the pathogenesis of Wilson's disease. Additionally, ATP7B has become a potential target for treating Wilson's disease, and the development of therapies targeting ATP7B holds promise for improving the treatment outcomes of this condition.
● ATP7B Recombinant Monoclonal Antibody CSB-RA175460A0HU
Validated Data
IHC image of CSB-RA175460A0HU diluted at 1:50 and staining in paraffin-embedded human brain tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.19% DAB
IHC image of CSB-RA175460A0HU diluted at 1:50 and staining in paraffin-embedded human ovarian cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.19% DAB
Immunofluorescence staining of HepG2 with CSB-RA175460A0HU at 1:20, counter-stained with DAPI. The cells were fixed in 4% formaldehyde and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 494-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
Overlay Peak curve showing HepG2 cells stained with CSB-RA175460A0HU (red line) at 1:50. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1µg/1*106cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated Goat Anti-rabbit IgG(H+L) at 1:200 dilution for 35min at 4℃.Control antibody (green line) was rabbit IgG (1µg/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.
The following ATP7B reagents supplied by CUSABIO are manufactured under a strict quality control system. Multiple applications have been validated and solid technical support is offered.
ATP7B Antibodies for Homo sapiens (Human)
Code | Product Name | Species Reactivity | Application |
---|---|---|---|
CSB-PA002415ESR2HU | ATP7B Antibody |
Human | ELISA, IHC |
CSB-PA030103 | ATP7B Antibody |
Human,Mouse,Rat | IHC, IF, ELISA |
CSB-RA175460A0HU | ATP7B Recombinant Monoclonal Antibody |
Human | ELISA, IHC, IF, FC |
CSB-RA002415MA1HU | ATP7B Recombinant Monoclonal Antibody |
Human | ELISA, IF, FC |
ATP7B Proteins for Rattus norvegicus (Rat)
Code | Product Name | Source |
---|---|---|
CSB-YP714496RA CSB-EP714496RA CSB-BP714496RA CSB-MP714496RA CSB-EP714496RA-B |
Recombinant Rat Copper-transporting ATPase 2 (Atp7b), partial |
Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
ATP7B Proteins for Mus musculus (Mouse)
Code | Product Name | Source |
---|---|---|
CSB-YP723743MO CSB-EP723743MO CSB-BP723743MO CSB-MP723743MO CSB-EP723743MO-B |
Recombinant Mouse Copper-transporting ATPase 2 (Atp7b), partial |
Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
ATP7B Proteins for Ovis aries (Sheep)
Code | Product Name | Source |
---|---|---|
CSB-YP896432SH CSB-EP896432SH CSB-BP896432SH CSB-MP896432SH CSB-EP896432SH-B |
Recombinant Sheep Copper-transporting ATPase 2 (ATP7B), partial |
Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
ATP7B Proteins for Homo sapiens (Human)
Code | Product Name | Source |
---|---|---|
CSB-YP002415HU CSB-BP002415HU CSB-MP002415HU CSB-EP002415HU-B |
Recombinant Human Copper-transporting ATPase 2 (ATP7B), partial |
Yeast Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |