ATP7A anticorps

N° du produit ABIN2192174

Détail du produit anti-ATP7A anticorps

Antigène: ATP7A (ATPase, Cu++ Transporting, alpha Polypeptide (ATP7A))

Reactivité: Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp ATP7A est non-conjugé

Application: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Flow Cytometry (FACS)

Stérilité: 0.2 μm filtered

Information d'application

Indications d'application: For immunohistochemistry, and Western blotting, dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions. The typical starting working dilution is 1:50. For functional studies, in vitro dilutions have to be optimized in user's experimental setting. Positive mouse pituitaries control

Restrictions: For Research Use only

Stockage

Buffer: PBS, containing 0.1 % bovine serum albumin and 0.02 % sodium azide.

Agent conservateur: Sodium azide

Précaution d'utilisation: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Stock: 4 °C

Stockage commentaire: Product should be stored at 4 °C. Under recommended storage conditions, product is stable for at least one year. The exact expiry date is indicated on the label.

Références pour anticorps anti-ATP7A (ABIN2192174)

Steveson, Ciccotosto, Ma, Mueller, Mains, Eipper: "Menkes protein contributes to the function of peptidylglycine alpha-amidating monooxygenase." dans: Endocrinology, Vol. 144, Issue 1, pp. 188-200, (2002) (PubMed).

Détails sur ATP7A

Antigène: ATP7A (ATPase, Cu++ Transporting, alpha Polypeptide (ATP7A))

Abstract: ATP7A Produits

Synonymes: anticorps ATP7A, anticorps cal, anticorps wu:fc43e01, anticorps zgc:153422, anticorps zgc:158633, anticorps DDBDRAFT_0218568, anticorps DDBDRAFT_0235190, anticorps DDB_0218568, anticorps DDB_0235190, anticorps atpase, anticorps Atp7a, anticorps kal, anticorps atp7a, anticorps DSMAX, anticorps MK, anticorps MNK, anticorps SMAX3, anticorps Blo, anticorps DXHXS1608e, anticorps I14, anticorps Mo, anticorps blotchy, anticorps br, anticorps brindled, anticorps mottled, anticorps Mnk, anticorps ATPase copper transporting alpha, anticorps ATPase, Cu++ transporting, alpha polypeptide, anticorps P-type ATPase, anticorps ATP synthase subunit a, anticorps copper-transporting ATPase 1, anticorps ATP7A, anticorps atp7a, anticorps LOC100049514, anticorps Atp7a, anticorps LOC412379

Sujet: Rabbit polyclonal antibody CT77 reacts with mouse and rat ATP7A. Copper is essential for human health and copper imbalance is a key factor in the aetiology and pathology of several neurodegenerative diseases. Copper uptake into cells is thought to be mediated by the plasma membrane protein CTR1. Metallochaperones also bind copper and target it to specific destinations within the cell. ATOX1 (HAH1) transfers copper to the copper-ATPases. Copper-transporting ATPases (Cu-ATPases) ATP7A and ATP7B are evolutionarily conserved polytopic membrane proteins with essential roles in human physiology. The Cu-ATPases are expressed in most tissues, and their transport activity is crucial for central nervous system development, liver function, connective tissue formation, and many other physiological processes. These proteins have a dual role in cells, namely to provide sufficient amounts of essential intracellular copper and to mediate the excretion of excess of intracellular copper. ATP7A and ATP7B are members of a large family of P-type ATPases that are energy-utilizing membrane proteins functioning as cation pumps. They are called 'P-type' ATPases, as they form a phosphorylated intermediate during the transport of cations across a membrane. The domains involved in the catalytic cycle of the protein are the nucleotide-binding domain (N-domain), phosphorylation domain (P-domain), and activation domain (A-domain). ATP7A is anchored to a membrane through eight hydrophobic transmembrane domains, which form a channel for copper translocation through the membrane. At the N-terminus ATP7A has six metal-binding domains (MBD1-6) each with a consensus MTXCXXC motif. Copper binds to these domains in the reduced form, Cu(I). It is assumed that the two MBDs (MBD5 and MBD6) closest to the transmembrane domains are important for the functional activity of the protein, and at least one of these two sites is necessary for normal function of the protein. The first four metal-binding domains (MBD1-4) are thought to have a regulatory function. Interaction between ATP7A and the copper chaperone ATOX1 occurs through these domains. ATP7A is expressed in almost every organ except the liver where ATP7B is predominantly expressed. In concordance with this, copper is incorporated in ceruloplasmin by ATP7B in hepatocytes, while ATP7A is in charge in most other cell types in transporting copper to tissue-specific enzymes. The malfunctioning of copper homeostasis is demonstrated in Menkes disease in which the ATP7A gene is defective. Menkes disease results in copper accumulation in intestinal cells, placenta, mammary tissue and the kidneys and deficiency in the brain, liver and serum. This leads to disrupted neurological and connective tissue development, causing mental retardation and neurodegeneration and usually results in early childhood death. Disturbances in copper homeostasis are also associated with neurodegenerative disorders such as Parkinson's and Alzheimer's disease, age-related macular degeneration and prion-related disease. Polyclonal antiserum CT77, raised against the C-terminal end of ATP7A, recognizes the full length protein. Immunogen Peptide (Asp1475-Leu1492) situated at the C-terminus of mouse (NM009726) and rat (NM052803) ATP7A

Pathways: Transition Metal Ion Homeostasis, Ribonucleoside Biosynthetic Process