Anti-Copper ATPase 1 (Menke's Disease Protein) Antibody (L60/4)

Name: Anti-Copper ATPase 1 (Menke's Disease Protein) Antibody
Brand: NeuroMab
SKU: 75-142
Price: 319.0 USD
Availability: InStock

4 Citations

Our Anti-Copper ATPase 1 (Menke's disease protein) mouse monoclonal primary antibody from NeuroMab is produced in-house from hybridoma clone L60/4. It is KO validated, detects human, mouse, and rat Copper ATPase 1 (Menke's disease protein), and is purified by Protein A chromatography. It is great for use in IHC, ICC, IP, WB.

Immunoblot of lysates from human skin fibroblasts from an ATP7A wild-type individual (ATP7A+/+, Lane 1) and three individuals with ATP7A null mutations (ATP7A-/-, Lanes 2-4) probed with L60/4 (top) and actin (bottom). Data courtesy of Stephanie Zlatic and Victor Faundez (Emory University). See reference 2017 Comstra et al., eLife (PMID 28355134) for further detail.

Adult rat hippocampus immunohistochemistry.

Species Reactivity

Human, Mouse, Rat

Applications

ICC, IHC, IP, WB

Host Species

Mouse

Available Conjugates

490

550

594

650

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Data Sheet

KO Validated

SKU: 75-142

Volume: 100 µL

Variant

Price:

$319.00

Quantity:

Product Details

Target

Copper ATPase 1 (Menke's disease protein)

Target Description

Copper-transporting ATPase 1 or ATPase Copper Transporting Alpha is encoded by the gene ATP7A and belongs to the cation transport ATPase (P-type) family. Copper-transporting ATPase 1 is a transmembrane enzyme that functions to provide copper to copper requiring proteins in the Golgi network and is also to remove excess copper across plasma membranes out of the cell. Copper-transporting ATPase 1 is found in most tissues except liver. Mutations in this gene are associated with Menkes disease, X-linked distal spinal muscular atrophy, and occipital horn syndrome.

Format

Purified by Protein A chromatography

Concentration

1 mg/mL

Clonality

Monoclonal

Clone

L60/4

Isotype

IgG2b

Applications

ICC, IHC, IP, WB

Host Species

Mouse

Gene Name

ATP7A MC1 MNK

Molecular Weight

180 kDa in rat brain membrane preparations

Antigen

Synthetic peptide amino acids 42-61 (cytoplasmic N- terminus) of human Copper-transporting ATPase 1 (accession number Q04656)

Species Reactivity

Human, Mouse, Rat

Antibody Registry ID

AB_2062824

Storage

Aliquot and store at ≤ -20°C for long term storage. For short term storage, store at 2-8°C. For maximum recovery of product, centrifuge the vial prior to removing the cap.

Physical State

Liquid

Production Notes

Produced by in vitro bioreactor culture of hybridoma line followed by Protein A affinity chromatography. Purified mAbs are >90% specific antibody.

Buffer

10 mM Tris, 50 mM Sodium Chloride, 0.065% Sodium Azide pH 7.68

Conjugate

Unconjugated

Specificity

No cross-reactivity reported

Quality Control

Each new lot of antibody is quality control tested on cells overexpressing target protein and confirmed to give the expected staining pattern.

Usage Statement

These antibodies are to be used as research laboratory reagents and are not for use as diagnostic or therapeutic reagents in humans.

Country of Origin

United States

Expiration

24 months from date of receipt

Shipping

Shipped on ice packs

Synonyms

Copper-transporting ATPase 1 (EC 3.6.3.54) (Copper pump 1) (Menkes disease-associated protein)

UniProt Details

UniProt (Human): Q04656
UniProt (Immunogen Species): Q04656

Tsai CY, Liebig JK, Tsigelny IF, Howell SB. (2015), 'The copper transporter 1 (CTR1) is required to maintain the stability of copper transporter 2 (CTR2)..' Metallomics. 10.1039/c5mt00131e.
Bonnemaison ML, Bäck N, Duffy ME, Ralle M, Mains RE, Eipper BA. (2015), 'Adaptor Protein-1 Complex Affects the Endocytic Trafficking and Function of Peptidylglycine α-Amidating Monooxygenase, a Luminal Cuproenzyme..' The Journal of Biological Chemistry. 10.1074/jbc.M115.641027.
Gaier ED, Miller MB, Ralle M, Aryal D, Wetsel WC, Mains RE, Eipper BA. (2013), 'Peptidylglycine α-amidating monooxygenase heterozygosity alters brain copper handling with region specificity..' Journal of Neurochemistry. 10.1111/jnc.12438.
Abada PB, Larson CA, Manorek G, Adams P, Howell SB. (2012), 'Sec61β controls sensitivity to platinum-containing chemotherapeutic agents through modulation of the copper-transporting ATPase ATP7A..' Molecular Pharmacology. 10.1124/mol.112.079822.

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Anti-Copper ATPase 1 (Menke's Disease Protein) Antibody (L60/4)

SKU: 75-142

Product Details

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