Mouse Copper-Transporting ATPase2 Monoclonal Antibody | anti-ATP7B antibody

Copper Transporting ATPase 2 Antibody: ATTO 594

Cat. Num. AAA800491

• Gene Names: ATP7B; WD; PWD; WC1; WND
• Reactivity: Human, Mouse, Rat
• Applications: Immunohistochemistry, Western Blot, Immunoprecipitation
• Synonyms: Copper-Transporting ATPase2; Monoclonal Antibody; Copper Transporting ATPase 2 Antibody: ATTO 594; ATP7B; ATPase Cu++ transporting beta polypeptide; ATPase Cu(2+) transporting beta polypeptide; Copper pump 2; Copper transporting ATPase 2; PWD; Toxic milk; tx; WC1; WD; Wilson disease associated protein; WND; WND/140 kDa; anti-ATP7B antibody

• Host: Mouse
• Reactivity: Human, Mouse, Rat
• Clonality: Monoclonal
• Isotype: IgG1
• Clone Number: S62-29
• Form/Format: Protein G Purified
• Concentration: 1mg/mL (varies by lot)
• Sequence Length: 1465

• Applicable Applications for anti-ATP7B antibody: Immunohistochemistry (IHC), Western Blot (WB), Immunoprecipitation (IP)
• Application Notes: 1:1000 (WB)
• Conjugate: ATTO 594
• Storage Buffer: PBS pH7.4, 50% glycerol, 0.09% sodium azide
• Preparation and Storage: -20 degree C

Immunocytochemistry (ICC)

(Immunocytochemistry/Immunofluorescence analysis using Mouse Anti-Copper Transporting ATPase 2 Monoclonal Antibody, Clone S62-29 . Tissue: NIH 3T3 (Mouse Fibroblast cell line). Species: Mouse. Fixation: 4% Formaldehyde for 15 min at RT. Primary Antibody: Mouse Anti-Copper Transporting ATPase 2 Monoclonal Antibody at 1:100 for 60 min at RT. Secondary Antibody: Goat Anti-Mouse ATTO 488 at 1:200 for 60 min at RT. Counterstain: Phalloidin Texas Red F-Actin stain; DAPI (blue) nuclear stain at 1:1000, 1:5000 for 60 min at RT, 5 min at RT. Localization: Cytoplasm . Magnification: 60X.)

Western Blot (WB)

(Western Blot analysis of Rat Brain Membrane showing detection of ~160 kDa Copper Transporting ATPase 2 protein using Mouse Anti-Copper Transporting ATPase 2 Monoclonal Antibody, Clone S62-29 . Lane 1: Molecular Weight Ladder (MW). Lane 2: Rat Brain Membrane cell lysate. Load: 20 ug. Block: 2% BSA and 2% Skim Milk in 1X TBST. Primary Antibody: Mouse Anti-Copper Transporting ATPase 2 Monoclonal Antibody at 1:1000 for 16 hours at 4 degree C. Secondary Antibody: Goat Anti-Mouse IgG: HRP at 1:100 for 60 min at RT. Color Development: ECL solution for 6 min in RT. Predicted/Observed Size: ~160 kDa.)

Related Product Information for anti-ATP7B antibody

Background Info: Detects ~160kDa in rat brain membrane preparations

Scientific Background: The copper efflux transporters ATP7A and ATP7B sequester intracellular copper into the vesicular secretory pathway for export from the cell. ATP7b is an important protein for copper transport and elimination of excess copper from the body. ATP7b transports metals in and out of cells using ATP. There are 3 known isoforms of the ATP7b gene; A is found in the liver, kidney, and brain, the shorter form B is found in brain tissue, and the third isoform, known as WND/140 KDA is found in mitochondria. Mutations in the ATP7b gene can cause Wilson's disease, an inherited disorder causing copper poisoning in the brain and liver, characterized by neurological symptoms and hepatic damage.

Product Categories/Family for anti-ATP7B antibody

Transporters

References

1. Tanzi R.E., et al. (1993) Nature Genetics. 5: 344-350. 2. Ghr/nlm.gov/gene/ATP7B

NCBI and Uniprot Product Information

• NCBI GI #: 55743071
• NCBI GeneID: 540
• NCBI Accession #: NP_000044.2
• NCBI GenBank Nucleotide #: NM_000053.3
• UniProt Accession #: B7ZLR4; P35670
• Molecular Weight: 151,875 Da
• NCBI Official Full Name: copper-transporting ATPase 2 isoform a
• NCBI Official Synonym Full Names: ATPase, Cu++ transporting, beta polypeptide
• NCBI Official Symbol: ATP7B
• NCBI Official Synonym Symbols: WD; PWD; WC1; WND
• NCBI Protein Information: copper-transporting ATPase 2; copper pump 2; Wilson disease-associated protein; ATPase, Cu(2+)- transporting, beta polypeptide
• UniProt Protein Name: ATP7B protein
• UniProt Gene Name: ATP7B
• UniProt Entry Name: B7ZLR4_HUMAN

NCBI 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 functions as a monomer, exporting 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 (WD). [provided by RefSeq, Jul 2008]

Uniprot Description

ATP7B: Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile. Monomer. Interacts with COMMD1/MURR1. Most abundant in liver and kidney and also found in brain. Isoform 2 is expressed in brain but not in liver. The cleaved form WND/140 kDa is found in liver cell lines and other tissues. Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Transporter; Membrane protein, multi-pass; EC 3.6.3.54; Hydrolase; Transporter, ion channel; Vesicle

Chromosomal Location of Human Ortholog: 13q14.3

Cellular Component: Golgi membrane; membrane; mitochondrion; perinuclear region of cytoplasm; integral to plasma membrane; basolateral plasma membrane; late endosome; cytoplasmic membrane-bound vesicle; trans-Golgi network

Molecular Function: protein binding; copper ion binding; copper-exporting ATPase activity; ATP binding

Biological Process: lactation; cellular copper ion homeostasis; response to copper ion; metabolic process; cellular zinc ion homeostasis; sequestering of calcium ion; copper ion import; copper ion transport; transmembrane transport; copper ion export; intracellular copper ion transport

Disease: Wilson Disease