Key features and details
- Chicken polyclonal to ATP7A
- Reacts with: Human
- Isotype: IgY
Product nameAnti-ATP7A antibody
See all ATP7A primary antibodies
DescriptionChicken polyclonal to ATP7A
Species reactivityReacts with: Human
Predicted to work with: Rat
Recombinant fragment, corresponding to amino acids 1407-1500 of Human ATP7A.
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Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Storage bufferConstituent: PBS
Concentration information loading...
PurityImmunogen affinity purified
Purification notesPeptide immunogen affinity column
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
ab13995 has been referenced in 9 publications.
- Miller KA et al. Antibiotic treatment affects the expression levels of copper transporters and the isotopic composition of copper in the colon of mice. Proc Natl Acad Sci U S A 116:5955-5960 (2019). PubMed: 30850515
- Kakuda M et al. Copper ions are novel therapeutic agents for uterine leiomyosarcoma. Am J Obstet Gynecol N/A:N/A (2019). PubMed: 31351063
- Skjørringe T et al. Characterization of ATP7A missense mutants suggests a correlation between intracellular trafficking and severity of Menkes disease. Sci Rep 7:757 (2017). WB, ICC/IF ; Human . PubMed: 28389643
- Fieten H et al. The Menkes and Wilson disease genes counteract in copper toxicosis in Labrador retrievers: a new canine model for copper-metabolism disorders. Dis Model Mech 9:25-38 (2016). WB . PubMed: 26747866
- Chen DB et al. Penicillamine increases free copper and enhances oxidative stress in the brain of toxic milk mice. PLoS One 7:e37709 (2012). WB ; Mouse . PubMed: 22629446
- Ip V et al. Differential expression of ATP7A, ATP7B and CTR1 in adult rat dorsal root ganglion tissue. Mol Pain 6:53 (2010). WB . PubMed: 20836889
- Bohlken A et al. ATP7A is a novel target of retinoic acid receptor beta2 in neuroblastoma cells. Br J Cancer 100:96-105 (2009). WB ; Human . PubMed: 19127267
- Paulsen M et al. Evidence that translation reinitiation leads to a partially functional Menkes protein containing two copper-binding sites. Am J Hum Genet 79:214-29 (2006). PubMed: 16826513
- Møller LB et al. Identification and analysis of 21 novel disease-causing amino acid substitutions in the conserved part of ATP7A. Hum Mutat 26:84-93 (2005). PubMed: 15981243