其他名称： ATP7B; PWD; WC1; WND; Copper-transporting ATPase 2; Copper pump 2; Wilson disease-associated protein
分子量： 157kD
功能： catalytic activity:ATP + H(2)O + Cu(2+)(In) = ADP + phosphate + Cu(2+)(Out).,disease:Defects in ATP7B are the cause of Wilson disease (WD) [MIM:277900]. WD is an autosomal recessive disorder of copper metabolism in which copper cannot be incorporated into ceruloplasmin in liver, and cannot be excreted from the liver into the bile. Copper accumulates in the liver and subsequently in the brain and kidney. The disease is characterized by neurologic manifestations and signs of cirrhosis.,function:Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.,online information:Wilson's disease website,PTM:Isoform 1 may be proteolytically cleaved at the N-terminus to produce the WND/140 kDa form.,similarity:Belongs to the cation transport ATPase (P-type) family.,similarity:Belongs to the cation transport ATPase (P-type) family. Type IB subfamily.,similarity:Contains 6 HMA domains.,subcellular location:Predominantly found in the trans-Golgi network (TGN). Not redistributed to the plasma membrane in response to elevated copper levels.,subunit:Monomer. Interacts with COMMD1/MURR1.,tissue specificity:Most abundant in liver and kidney and also found in brain. Isoform 2 is expressed in brain but not in liver. The cleaved form WND/140 kDa is found in liver cell lines and other tissues.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Golgi apparatus, trans-Golgi network membrane ; Multi-pass membrane protein . Late endosome . Predominantly found in the trans-Golgi network (TGN). Localized in the trans-Golgi network under low copper conditions, redistributes to cytoplasmic vesicles when cells are exposed to elevated copper levels, and then recycles back to the trans-Golgi network when copper is removed (PubMed:10942420). .; [Isoform 1]: Golgi apparatus membrane ; Multi-pass membrane protein .; [Isoform 2]: Cytoplasm .; [WND/140 kDa]: Mitochondrion .
组织表达： Most abundant in liver and kidney and also found in brain. Isoform 2 is expressed in brain but not in liver. The cleaved form WND/140 kDa is found in liver cell lines and other tissues.
其他名称： ATP7B; PWD; WC1; WND; Copper-transporting ATPase 2; Copper pump 2; Wilson disease-associated protein
分子量： 157kD
功能： catalytic activity:ATP + H(2)O + Cu(2+)(In) = ADP + phosphate + Cu(2+)(Out).,disease:Defects in ATP7B are the cause of Wilson disease (WD) [MIM:277900]. WD is an autosomal recessive disorder of copper metabolism in which copper cannot be incorporated into ceruloplasmin in liver, and cannot be excreted from the liver into the bile. Copper accumulates in the liver and subsequently in the brain and kidney. The disease is characterized by neurologic manifestations and signs of cirrhosis.,function:Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.,online information:Wilson's disease website,PTM:Isoform 1 may be proteolytically cleaved at the N-terminus to produce the WND/140 kDa form.,similarity:Belongs to the cation transport ATPase (P-type) family.,similarity:Belongs to the cation transport ATPase (P-type) family. Type IB subfamily.,similarity:Contains 6 HMA domains.,subcellular location:Predominantly found in the trans-Golgi network (TGN). Not redistributed to the plasma membrane in response to elevated copper levels.,subunit:Monomer. Interacts with COMMD1/MURR1.,tissue specificity:Most abundant in liver and kidney and also found in brain. Isoform 2 is expressed in brain but not in liver. The cleaved form WND/140 kDa is found in liver cell lines and other tissues.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Golgi apparatus, trans-Golgi network membrane ; Multi-pass membrane protein . Late endosome . Predominantly found in the trans-Golgi network (TGN). Localized in the trans-Golgi network under low copper conditions, redistributes to cytoplasmic vesicles when cells are exposed to elevated copper levels, and then recycles back to the trans-Golgi network when copper is removed (PubMed:10942420). .; [Isoform 1]: Golgi apparatus membrane ; Multi-pass membrane protein .; [Isoform 2]: Cytoplasm .; [WND/140 kDa]: Mitochondrion .
组织表达： Most abundant in liver and kidney and also found in brain. Isoform 2 is expressed in brain but not in liver. The cleaved form WND/140 kDa is found in liver cell lines and other tissues.
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