Anti-ATP7A antibody [L60/4] (ab131400)
Key features and details
- Mouse monoclonal [L60/4] to ATP7A
- Suitable for: ICC/IF, Flow Cyt
- Reacts with: Mouse, Human
- Isotype: IgG2b
Overview
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Product name
Anti-ATP7A antibody [L60/4]
See all ATP7A primary antibodies -
Description
Mouse monoclonal [L60/4] to ATP7A -
Host species
Mouse -
Tested applications
Suitable for: ICC/IF, Flow Cytmore details -
Species reactivity
Reacts with: Mouse, Human
Predicted to work with: Chinese hamster -
Immunogen
Synthetic peptide corresponding to Human ATP7A aa 42-61.
Sequence:SLEEKNATIIYDPKLQTPKT
Database link: Q04656 -
Positive control
- ICC/IF: NIH/3T3 cells. Flow Cyt: HT1080 cells.
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General notes
The clone number has been updated from S60-4 to L60/4, both clone numbers name the same antibody clone.
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Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at -20°C. -
Storage buffer
Preservative: 0.09% Sodium azide
Constituents: PBS, 50% Glycerol (glycerin, glycerine) -
Concentration information loading...
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Purity
Protein G purified -
Clonality
Monoclonal -
Clone number
L60/4 -
Isotype
IgG2b -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Positive Controls
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Recombinant Protein
Applications
The Abpromise guarantee
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.
Application | Abreviews | Notes |
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ICC/IF |
1/100.
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Flow Cyt |
Use 0.1µg for 106 cells.
ab170192 - Mouse monoclonal IgG2b, is suitable for use as an isotype control with this antibody. |
Notes |
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ICC/IF
1/100. |
Flow Cyt
Use 0.1µg for 106 cells. ab170192 - Mouse monoclonal IgG2b, is suitable for use as an isotype control with this antibody. |
Target
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Function
May 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 specificity
Found 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 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.
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 similarities
Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily.
Contains 6 HMA domains. -
Domain
The 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 localization
Endoplasmic 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
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Database links
- Entrez Gene: 538 Human
- Entrez Gene: 11977 Mouse
- Omim: 300011 Human
- SwissProt: Q04656 Human
- SwissProt: Q64430 Mouse
- Unigene: 496414 Human
- Unigene: 254297 Mouse
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Alternative names
- ATP 7A antibody
- ATP7A antibody
- ATP7A_HUMAN antibody
see all
Images
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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).
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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.
Protocols
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.
Datasheets and documents
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SDS download
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Datasheet download
References (3)
ab131400 has been referenced in 3 publications.
- Gao W et al. Elesclomol induces copper-dependent ferroptosis in colorectal cancer cells via degradation of ATP7A. Mol Oncol 15:3527-3544 (2021). PubMed: 34390123
- 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