Key features and details
- Mouse monoclonal [L60/4] to ATP7A
- Suitable for: ICC/IF, Flow Cyt
- Reacts with: Mouse, Human
- Isotype: IgG2b
Product nameAnti-ATP7A antibody [L60/4]
See all ATP7A primary antibodies
DescriptionMouse monoclonal [L60/4] to ATP7A
Tested applicationsSuitable for: ICC/IF, Flow Cytmore details
Species reactivityReacts with: Mouse, Human
Predicted to work with: Chinese hamster
- ICC/IF: NIH/3T3 cells. Flow Cyt: HT1080 cells.
The clone number has been updated from S60-4 to L60/4, both clone numbers name the same antibody clone.
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In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.
We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.
Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.
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Storage instructionsShipped at 4°C. Store at -20°C.
Storage bufferConstituents: 49% PBS, 50% Glycerol (glycerin, glycerine)
Concentration information loading...
PurityProtein G purified
Our Abpromise guarantee covers the use of ab131400 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|Flow Cyt||Use 0.1µg for 106 cells.
ab170192 - Mouse monoclonal IgG2b, is suitable for use as an isotype control with this antibody.
FunctionMay supply copper to copper-requiring proteins within the secretory pathway, when localized in the trans-Golgi network. Under conditions of elevated extracellular copper, it relocalized to the plasma membrane where it functions in the efflux of copper from cells.
Tissue specificityFound in most tissues except liver. Isoform 3 is widely expressed including in liver cell lines. Isoform 1 is expressed in fibroblasts, choriocarcinoma, colon carcinoma and neuroblastoma cell lines. Isoform 2 is expressed in fibroblasts, colon carcinoma and neuroblastoma cell lines.
Involvement in diseaseDefects 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.
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 diarrhea and genitourinary defects. The skeletal abnormalities included occipital horns, short, broad clavicles, deformed radii, ulnae and humeri, narrowing of the rib cage, undercalcified long bones with thin cortical walls and coxa valga.
Defects in ATP7A are a cause of distal spinal muscular atrophy X-linked type 3 (DSMAX3) [MIM:300489]. DSMAX3 is a neuromuscular disorder. Distal spinal muscular atrophy, also known as distal hereditary motor neuronopathy, represents a heterogeneous group of neuromuscular disorders caused by selective degeneration of motor neurons in the anterior horn of the spinal cord, without sensory deficit in the posterior horn. The overall clinical picture consists of a classical distal muscular atrophy syndrome in the legs without clinical sensory loss. The disease starts with weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs. Later on, weakness and atrophy may expand to the proximal muscles of the lower limbs and/or to the distal upper limbs.
Sequence similaritiesBelongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily.
Contains 6 HMA domains.
DomainThe C-terminal di-leucine, 1487-Leu-Leu-1488, is an endocytic targeting signal which functions in retrieving recycling from the plasma membrane to the TGN. Mutation of the di-leucine signal results in the accumulation of the protein in the plasma membrane.
Cellular localizationEndoplasmic reticulum; Cytoplasm > cytosol and Golgi apparatus > trans-Golgi network membrane. Cell membrane. Cycles constitutively between the trans-Golgi network (TGN) and the plasma membrane. Predominantly found in the TGN and relocalized to the plasma membrane in response to elevated copper levels.
- Information by UniProt
- ATP 7A antibody
- ATP7A antibody
- ATP7A_HUMAN antibody
NIH/3T3 (Mouse embryo fibroblast cell line) cells labeling ATP7A using ab131400 at 1/100 dilution in ICC/IF. Cells were fixed using 4% formaldehyde for 15 minutes at room temperature. Incubation with primary antibody was performed for 1 hour at room temperature. Secondary antibody used was a goat anti-mouse ATTO 488 (green) at 1/200 dilution for 1 hour at room temperature. Counterstained with Phalloidin Texas Red F-actin stain. Nuclei were stained with DAPI (blue).
Overlay histogram showing HT1080 cells stained with ab131400 (red line). The cells were fixed with 4% paraformaldehyde (10 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab131400, 0.1μg/1x106 cells) for 30 min at 22°C. The secondary antibody used was Alexa Fluor® 488 goat anti-mouse IgG (H+L) (ab150113) at 1/2000 dilution for 30 min at 22°C. Isotype control antibody (black line) was mouse IgG2b [PLPV219] (ab91366, 1μg/1x106 cells) used under the same conditions. Unlabelled sample (blue line) was also used as a control. Acquisition of >5,000 events were collected using a 20mW Argon ion laser (488nm) and 525/30 bandpass filter. This antibody gave a positive signal in HT1080 cells fixed with 80% methanol (5 min)/permeabilized with 0.1% PBS-Tween for 20 min used under the same conditions.
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
ab131400 has been referenced in 2 publications.
- Li K et al. The loss of copper is associated with the increase in copper metabolism MURR domain 1 in ischemic hearts of mice. Exp Biol Med (Maywood) 243:780-785 (2018). PubMed: 29763364
- Arciello M et al. Core domain mutant Y220C of p53 protein has a key role in copper homeostasis in case of free fatty acids overload. Biometals 28:1017-29 (2015). WB . PubMed: 26438057