Catalog Number:
E20-70412Amount:
100ulPrice:
225$Purification:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.Immunogen:
The antiserum was produced against synthesized peptide derived from human ATP7B. AA range:161-210Source:
Polyclonal, RabbitGene Name:
ATP7BDilution:
Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/5000. Not yet tested in other applications.
Background:
ATPase copper transporting beta(ATP7B) Homo sapiens 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],Protein Name:
Copper-transporting ATPase 2Human Gene Id:
540Human Swiss Prot No:
P35670Mouse Gene Id:
11979Mouse Swiss Prot No:
Q64446Rat Swiss Prot No:
Q64535Specificity:
ATP7B Polyclonal Antibody detects endogenous levels of ATP7B protein.Formulation:
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.Concentration:
1 mg/mlStorage Stability:
-20°C/1 yearOther Name:
ATP7B; PWD; WC1; WND; Copper-transporting ATPase 2; Copper pump 2; Wilson disease-associated proteinMolecularWeight(Da):
157334Function:
catalytic activity:ATP + H(2)O + Cu(2+)(In) = ADP + phosphate + Cu(2+)(Out).,disease:Defects in ATP7B are the cause of Wilson disease (WD) [MIM:277900]. WD is an autosomal recessive disorder of copper metabolism in which copper cannot be incorporated into ceruloplasmin in liver, and cannot be excreted from the liver into the bile. Copper accumulates in the liver and subsequently in the brain and kidney. The disease is characterized by neurologic manifestations and signs of cirrhosis.,function:Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.,online information:Wilson's disease website,PTM:Isoform 1 may be proteolytically cleaved at the N-terminus to produce the WND/140 kDa form.,similarity:Belongs to the cation transport ATPase (P-type) family.,similarity:Belongs to the cation transport ATPase (P-type) family. Type IB subfamily.,similaSubcellular Location:
Golgi membrane,mitochondrion,late endosome,trans-Golgi network,integral component of plasma membrane,membrane,integral component of membrane,basolateral plasma membrane,cytoplasmic vesicle,trans-Golgi network membrane,perinuclear rExpression:
Brain,Liver,Pooled,
Catalog Number:
E011009-1Amount:
50μg/50μlReactivity:
Human, Mouse, RatApplications:
IHC: 1:50~1:100Swiss-Prot No.:
P60484Price:
145$
Catalog Number:
E011009-2Amount:
100μg/100μlReactivity:
Human, Mouse, RatApplications:
IHC: 1:50~1:100Swiss-Prot No.:
P60484Price:
225$
Catalog Number:
E011013-1Amount:
50μg/50μlReactivity:
Human, Mouse, RatApplications:
WB: 1:500~1:1000 IF:1:100~1:200Swiss-Prot No.:
Q04206Price:
145$
Catalog Number:
E011013-2Amount:
100μg/100μlReactivity:
Human, Mouse, RatApplications:
WB: 1:500~1:1000 IF:1:100~1:200Swiss-Prot No.:
Q04206Price:
225$
Catalog Number:
E011020-1Amount:
50μg/50μlReactivity:
HumanApplications:
IF:1:100~1:200Swiss-Prot No.:
Q04864Price:
145$