ATP7A Antikörper

Produktnummer ABIN2192174

Produktdetails anti-ATP7A Antikörper

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

Reaktivität: Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser ATP7A Antikörper ist unkonjugiert

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

Sterilität: 0.2 μm filtered

Anwendungsinformationen

Applikationshinweise: 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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

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

Konservierungsmittel: Sodium azide

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

Lagerung: 4 °C

Informationen zur Lagerung: 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.

Referenzen für anti-ATP7A Antikörper (ABIN2192174)

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

Details zu ATP7A

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

Abstract: ATP7A Produkte

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

Hintergrund: 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