Overview

  • Product name
  • Description
    Rabbit polyclonal to ATP7A
  • Tested applications
    Suitable for: ICC/IF, WB, IHC-Pmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Cat, Dog, Chimpanzee
  • Immunogen

    Synthetic peptide corresponding to Human ATP7A. Short peptide sequence used to raise this antibody is 100% homologous to isoform 4 (1500aa, 163kDa), 1 (1514aa, 165kDa), 2 (1581aa, 172kDa), 5 (1422aa, 154kDa) of human ATP7A
    Database link: Q04656

  • Positive control
    • WB: HepG2 cell lysate. IHC-P: Human kidney

Properties

Applications

Our Abpromise guarantee covers the use of ab42486 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
ICC/IF Use a concentration of 5 µg/ml.
WB Use a concentration of 0.5 µg/ml. Predicted molecular weight: 30 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
IHC-P Use at an assay dependent concentration.

Target

  • 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
  • Database links
  • Alternative names
    • ATP 7A antibody
    • ATP7A antibody
    • ATP7A_HUMAN antibody
    • ATPase copper transporting alpha polypeptide antibody
    • ATPase Cu++ transporting alpha polypeptide (Menkes syndrome) antibody
    • ATPase Cu++ transporting alpha polypeptide antibody
    • Copper pump 1 antibody
    • Copper transporting ATPase 1 antibody
    • Copper-transporting ATPase 1 antibody
    • Cu++ transporting P type ATPase antibody
    • DSMAX antibody
    • FLJ17790 antibody
    • MC 1 antibody
    • MC1 antibody
    • Menkes disease associated protein antibody
    • Menkes disease-associated protein antibody
    • Menkes syndrome antibody
    • MK antibody
    • MNK antibody
    • OHS antibody
    • OTTHUMP00000062077 antibody
    • SMAX3 antibody
    see all

Anti-ATP7A antibody images

  • Antibody ab42486 (4.0-8.0 ug/ml) immunohistochemistry staining of paraffin embedded human kidney. Positive staining of epithelial cells of human renal tubules idicated by arrows.Antibody ab42486 (4.0-8.0 ug/ml) immunohistochemistry staining of paraffin embedded human kidney. Positive staining of epithelial cells of human renal tubules idicated by arrows.
  • ICC/IF image of ab42486 stained Mcf7 cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab42486, 5µg/ml) overnight at +4°C. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.

References for Anti-ATP7A antibody (ab42486)

This product has been referenced in:
  • Anandhan A  et al. Overexpression of alpha-synuclein at non-toxic levels increases dopaminergic cell death induced by copper exposure via modulation of protein degradation pathways. Neurobiol Dis 81:76-92 (2015). WB ; Rat . Read more (PubMed: 25497688) »

See 1 Publication for this product

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We are still currently investigating the issue with ab42486. We are reviewingthe targetof this antibody and the datasheet will beupdated as soonas possible.

We don not have any information about the expression of ATP7A in muscle, ho...

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I have received your request for the replacement of ab42486with ab131400. Please note that ab131400 is not currently in stock and should be delivered to you within one week, maximum 2.

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It looks likewrong information was entered for ab42486. My colleaguesin charge of the product datasheets are reviewing this problem and the websitewill beupdated as soon a...

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Thank you very much for contacting us and for reporting this inconsistency. I just wanted to let you know that I am still working on your inquiry. I have contacted the originator of this product for the information you requested, and I am awaiting a...

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Thank you for your enquiry. I can confirm there are in two different isoforms for this protein, The 30 KD protein is a much shorter alternate isoform that is listed in both the NCBI an SwissProt databases and is sometimes detected for ATP7A. ...

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