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ATP7A Polyclonal Antibody
Polyclonal, Rabbit
Price on request
Quantity
Ship worldwide. Contact order@enogene.com for ordering and bulk pricing or contact your local distributor.
Gene NameATP7A
Product Information
Protein NameCopper-transporting ATPase 1
Human Gene Id538
Human Swiss Prot NoQ04656
Mouse Gene Id11977
Mouse Swiss Prot NoQ64430
Rat Gene Id24941
Rat Swiss Prot NoP70705
SpecificityATP7A Polyclonal Antibody detects endogenous levels of ATP7A protein.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Concentration1 mg/ml
Storage Stability-20°C/1 year
Other NameATP7A; MC1; MNK; Copper-transporting ATPase 1; Copper pump 1; Menkes disease-associated protein
MolecularWeight(Da)163335/165110/172078/154357
Functioncatalytic activity：ATP + H(2)O + Cu(2+)(In) = ADP + phosphate + Cu(2+)(Out).,disease：Defects in ATP7A are the cause of Menkes disease (MNKD) [MIM：309400]; also known as kinky hair disease. MNKD is an X-linked recessive disorder of copper metabolism characterized by generalized copper deficiency. MNKD results in progressive neurodegeneration and connective-tissue disturbances： focal cerebral and cerebellar degeneration, early growth retardation, peculiar hair, hypopigmentation, cutis laxa, vascular complications and death in early childhood. The clinical features result from the dysfunction of several copper-dependent enzymes.,disease：Defects in ATP7A are the cause of occipital horn syndrome (OHS) [MIM：304150]; also known as X-linked cutis laxa. OHS is an X-linked recessive disorder of copper metabolism. Common features are unusual facial appearance, skeletal abnormalities, chronic diarrh
Subcellular Locationlate endosome,endoplasmic reticulum,Golgi apparatus,trans-Golgi network,cytosol,plasma membrane,integral component of plasma membrane,membrane,integral component of membrane,basolateral plasma membrane,trans-Golgi network transport
ExpressionBrain,Colon carcinoma,Endothelial cell,Fibroblast,Kidney,Neuroblastoma,
ImmunogenThe antiserum was produced against synthesized peptide derived from human ATP7A. AA range：591-640
DilutionImmunohistochemistry： 1/100 - 1/300. ELISA： 1/40000. Not yet tested in other applications.
PurificationThe antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
BackgroundATPase copper transporting alpha(ATP7A) Homo sapiens This gene encodes a transmembrane protein that functions in copper transport across membranes. This protein is localized to the trans Golgi network, where it is predicted to supply copper to copper-dependent enzymes in the secretory pathway. It relocalizes to the plasma membrane under conditions of elevated extracellular copper, and functions in the efflux of copper from cells. Mutations in this gene are associated with Menkes disease, X-linked distal spinal muscular atrophy, and occipital horn syndrome. Alternatively-spliced transcript variants have been observed. [provided by RefSeq, Aug 2013],
Conjugated Antibody
This product is also available conjugated. Format: ：E20-70411-Conjugate. Price: $485 / Size: 100μl.
：E20-70411-FITC$485 / 100μl：E20-70411-Biotin$485 / 100μl：E20-70411-HRP$485 / 100μl：E20-70411-AP$485 / 100μl：E20-70411-AF350$485 / 100μl：E20-70411-AF405M$485 / 100μl：E20-70411-AF488$485 / 100μl：E20-70411-AF514$485 / 100μl：E20-70411-AF532$485 / 100μl：E20-70411-AF555$485 / 100μl：E20-70411-AF568$485 / 100μl：E20-70411-AF405S$485 / 100μl：E20-70411-AF405L$485 / 100μl：E20-70411-AF546$485 / 100μl：E20-70411-AF594$485 / 100μl：E20-70411-AF610$485 / 100μl：E20-70411-AF635$485 / 100μl：E20-70411-AF647$485 / 100μl：E20-70411-AF680$485 / 100μl：E20-70411-AF700$485 / 100μl：E20-70411-AF750$485 / 100μl：E20-70411-AF790$485 / 100μl：E20-70411-APC$485 / 100μl：E20-70411-PE$485 / 100μl：E20-70411-Cy3$485 / 100μl：E20-70411-Cy5$485 / 100μl：E20-70411-Cy5.5$485 / 100μl：E20-70411-Cy7$485 / 100μl：E20-70411-PE-Cy5$485 / 100μl
Contact order@enogene.com to order conjugated formats. Magnetic Beads (1-30μm) conjugation is also available.
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