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| Catalog Code: | NB120-13995 |
| Product Name: | ATP7A Antibody |
| Product Description: | Guinea Pig Polyclonal anti-ATP7A |
| Clonality: | Polyclonal |
| ListPrice: | 295 |
| Concentration: | 1 mg/ml |
| Immunogen: | Recombinant fragment, corresponding to amino acids 1407-1500 of Human ATP7A. |
| Cross Reactivity: | Cross-reacts with Human, Mouse and Rat. Not yet tested in other species. |
| Packaging: | 0.05 mg Immunogen affinity purified Guinea Pig antisera. |
| Localization: | Golgi Apparatus and Plasma membrane |
| Storage: | Copper-transporting ATPase 1 is an integral membrane protein cycling constitutively between the trans-golgi network and the plasma membrane. It 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. Defects in ATP7A are the cause of Menkes syndrome ; also known as kinky hair disease, an X-linked recessive disorder. |
| Purity: | Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze thaw cycles. |
| Isotype: | Immunogen affinity purified |
| Host Name: | IgY |
| Buffer: | Guinea Pig |
| Application Summary: | PBS |
| Species Summary: | Western Blot 1/2000 |
| Alternate Names: | Hu, Mu, Rt, |
| Package Size: | anti-Copper transporting ATPase 1 antibody, anti-Copper pump 1 antibody |
| Preservative: | 0.05 mg |
| Homology: | No Preservative |
| Product References: | [1] Lane,C., Petris,M.J., Benmerah,A., Greenough,M. and Camakaris,J., et al. Studies on endocytic mechanisms of the menkes copper-translocating P-type ATPase (ATP7A; MNK). Endocytosis of the menkes protein. Biometals. 2004 Feb;17(1):87-98. [2] Pascale,M.C., Franceschelli,S., Moltedo,O., Belleudi,F., Torrisi,M.R., Bucci,C., La Fontaine,S., Mercer,J.F. and Leone,A. Endosomal trafficking of the Menkes copper ATPase ATP7A is mediated by vesicles containing the Rab7 and Rab5 GTPase proteins. Exp Cell Res. 2003 Dec 10;291(2):377-85. [3] Tumer,Z., Birk Moller,L. and Horn,N., et al. Screening of 383 unrelated patients affected with Menkes disease and finding of 57 gross deletions in ATP7A. Hum Mutat. 2003 Dec;22(6):457-64. [4] Jones,C.E., Daly,N.L., Cobine,P.A., Craik,D.J. and Dameron,C.T., et al. Structure and metal binding studies of the second copper binding domain of the Menkes ATPase. J Struct Biol. 2003 Sep;143(3):209-18. [5] Cobbold,C., Coventry,J., Ponnambalam,S. and Monaco,A.P., et al. The Menkes disease ATPase (ATP7A) is internalized via a Rac1-regulated, clathrin- and caveolae-independent pathway. Hum Mol Genet. 2003 Jul 1;12(13):1523-33. |
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