Gene Symbol: Atp7b
Description: ATPase copper transporting beta
Alias: Hts, PINA, copper-transporting ATPase 2, ATPase, Cu++ transporting, beta polypeptide (same as Wilson disease), PINA gene, promoter, copper pump 2, pineal night-specific ATPase, wilson disease-associated protein homolog
- A novel pineal night-specific ATPase encoded by the Wilson disease geneJ Borjigin
Department of Neuroscience, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
J Neurosci 19:1018-26. 1999We have identified a pineal night-specific ATPase (PINA), a novel splice variant of the ATP7B gene disrupted in Wilson disease (WD)...
- Liver ischemia and ischemia-reperfusion induces and trafficks the multi-specific metal transporter Atp7b to bile duct canaliculi: possible preferential transport of iron into bileJohn A Goss
Michael E DeBakey Department of Surgery, Liver Transplant Center Laboratory, Baylor College of Medicine, Houston, TX 77030, USA
Biol Trace Elem Res 122:26-41. 2008Both Atp7b (Wilson disease gene) and Atp7a (Menkes disease gene) have been reported to be trafficked by copper. Atp7b is trafficked to the bile duct canaliculi and Atp7a to the plasma membrane...
- NH2-terminal signals in ATP7B Cu-ATPase mediate its Cu-dependent anterograde traffic in polarized hepatic cellsY Guo
Department of Cell Biology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
Am J Physiol Gastrointest Liver Physiol 289:G904-16. 2005Cu is an essential cofactor of cellular proteins but is toxic in its free state. The hepatic Cu-ATPase ATP7B has two functions in Cu homeostasis: it loads Cu+ onto newly synthesized apoceruloplasmin in the secretory pathway, thereby ..
- Polarized sorting of the copper transporter ATP7B in neurons mediated by recognition of a dileucine signal by AP-1Shweta Jain
Cell Biology and Metabolism Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892
Mol Biol Cell 26:218-28. 2015..Here we report that polarized sorting of the Cu(2+) transporter ATP7B and the vesicle-SNARE VAMP4 to the somatodendritic domain of rat hippocampal neurons is mediated by recognition of ..
- Regulation of copper transport crossing brain barrier systems by Cu-ATPases: effect of manganese exposureXue Fu
School of Health Sciences, Purdue University, West Lafayette, Indiana 47907
Toxicol Sci 139:432-51. 2014Regulation of cellular copper (Cu) homeostasis involves Cu-transporting ATPases (Cu-ATPases), i.e., ATP7A and ATP7B. The question as to how these Cu-ATPases in brain barrier systems transport Cu, i.e...
- Subcellular targets of cisplatin cytotoxicity: an integrated viewSandra M Sancho-Martínez
Instituto de Investigación Biomédica de Salamanca IBSAL, 37007 Salamanca, Spain
Pharmacol Ther 136:35-55. 2012..Perspectives for the key aspects that need to be addressed by future investigation are also outlined...
- Effects of copper supplementation on copper absorption, tissue distribution, and copper transporter expression in an infant rat modelKathryn A Bauerly
Department of Nutrition, University of California Davis, One Shields Ave, Davis, California 95616, USA
Am J Physiol Gastrointest Liver Physiol 288:G1007-14. 2005..Intestine and liver were collected at days 10 and 20, and Cu concentration, Cu transporter-1 (Ctr1), Atp7A, Atp7B, and metallothionein (MT) mRNA and protein levels were measured. 67Cu absorption was measured at days 10 and 20...
- Clusterin and COMMD1 independently regulate degradation of the mammalian copper ATPases ATP7A and ATP7BStephanie Materia
Strategic Research Centre for Molecular and Medical Research, School of Life and Environmental Sciences, Deakin University, Burwood, Victoria 3125, Australia
J Biol Chem 287:2485-99. 2012ATP7A and ATP7B are copper-transporting P(1B)-type ATPases (Cu-ATPases) that are critical for regulating intracellular copper homeostasis...
- Mammary gland copper transport is stimulated by prolactin through alterations in Ctr1 and Atp7A localizationShannon L Kelleher
Department of Nutrition, University of California Davis, Davis, CA 95616, USA
Am J Physiol Regul Integr Comp Physiol 291:R1181-91. 2006..Three Cu-specific transporters (Ctr1, Atp7A, Atp7B) have been identified in the mammary gland; however, the integrated role they play in milk Cu secretion is not ..
- Aldo-keto reductase 1 family B7 is the gene induced in response to oxidative stress in the livers of Long-Evans Cinnamon ratsGuang Jia
Health Effects Research Team, National Institute for Environmental Studies, Tsukuba, Ibaraki 305 8506, Japan
Int J Oncol 29:829-38. 2006The Long-Evans Cinnamon (LEC) rat strain (Atp7b m/m), which accumulates copper in the liver due to mutations in the Atp7b gene, is a useful model for investigating the relationship between oxidative stress and hepatocarcinogenesis...
- The LEC rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease geneJ Wu
Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada
Nat Genet 7:541-5. 1994..We have cloned cDNAs for the rat gene (Atp7b) homologous to the human Wilson disease gene (ATP7B) and have used them to identify a partial deletion in the ..
- Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon ratY Yamaguchi
Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St Louis, MO 63110
Biochem J 301:1-4. 1994..These data therefore identify the Long-Evans Cinnamon rat as the first bona fide animal model of Wilson disease and suggest that this rat strain may be a valuable resource in the study of this genetic disorder...
- The gene responsible for LEC hepatitis, located on rat chromosome 16, is the homolog to the human Wilson disease geneN Sasaki
RIKEN Tsukuba Life Science Center, Institute of Physical and Chemical Research, Ibaraki, Japan
Biochem Biophys Res Commun 202:512-8. 1994We identified the rat homolog to the human Wilson disease (WD) gene as the gene responsible for hepatitis (hts) in the Long Evans Cinnamon (LEC) rat...
- Differential expression of ATP7A, ATP7B and CTR1 in adult rat dorsal root ganglion tissueVirginia Ip
Department of Pharmacology and Clinical Pharmacology, School of Medical Sciences, Faculty of Medical and Health Sciences, The University of Auckland, Auckland, New Zealand
Mol Pain 6:53. 2010ATP7A, ATP7B and CTR1 are metal transporting proteins that control the cellular disposition of copper and platinum drugs, but their expression in dorsal root ganglion (DRG) tissue and their role in platinum-induced neurotoxicity are ..
- Wilson protein expression, copper excretion and sweat production in sweat glands of Wilson disease patients and controlsMark Schaefer
Department of Gastroenterology and Infections Diseases, University of Heidelberg Medical School, INF 410, D 69120 Heidelberg, Germany
BMC Gastroenterol 8:29. 2008..It is the aim of this study to investigate Wilson protein expression in sweat glands and analysing its effects on copper excretion into sweat in controls and patients with Wilson disease...
- ATP7B copper-regulated traffic and association with the tight junctions: copper excretion into the bileSonia Hernandez
Centro de Biologia Molecular Severo Ochoa, Centro de Investigacion Biomedica en Red de Enfermedades Hepaticas y Digestivas CIBERehd, Consejo Superior de Investigaciones Cientificas, Madrid, Spain
Gastroenterology 134:1215-23. 2008The copper transporter ATP7B plays a central role in the elimination of excess copper by the liver into the bile, yet the site of its action remains controversial...
- A pineal regulatory element (PIRE) mediates transactivation by the pineal/retina-specific transcription factor CRXX Li
Department of Neuroscience, The Johns Hopkins University School of Medicine, 725 North Wolfe Street, Baltimore, MD 21205, USA
Proc Natl Acad Sci U S A 95:1876-81. 1998..and night-specific protein we recently identified, is produced as a truncated form of the Wilson disease gene (Atp7b) product...
- Abnormal hepatobiliary and circulating lipid metabolism in the Long-Evans Cinnamon rat model of Wilson's diseaseEmile Levy
Department of Nutrition, CHU Sainte Justine, Universite de Montreal, 3175 Cote Sainte Catherine, Montreal, Quebec, Canada
Life Sci 80:1472-83. 2007Long-Evans Cinnamon (LEC) rats exhibit a genetic defect in Atp7b gene, which is homologous to the human Wilson's disease gene, resulting in an inability to mobilize copper from the liver...
- Copper-transporting P-type adenosine triphosphatase (ATP7B) is expressed in human breast carcinomaAtsuko Kanzaki
Department of Pathology, Institute of Development, Aging and Cancer, Tohoku University, Aoba ku, Sendai 980 8575, Japan
Jpn J Cancer Res 93:70-7. 2002This is the first report to show that a copper-transporting P-type adenosine triphosphatase, ATP7B, is expressed in certain breast carcinomas, and a priori knowledge of its expression is important for the choice of therapy...
- Dominant-stable beta-catenin expression causes cell fate alterations and Wnt signaling antagonist expression in a murine granulosa cell tumor modelDerek Boerboom
Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, Texas 77030, USA
Cancer Res 66:1964-73. 2006..Together, these results suggest that misregulated Wnt/beta-catenin signaling alters the fate of granulosa cells and that the GCT that arise in Catnb(flox(ex3)/+);Amhr2(cre/+) mice result from the clonal expansion of metaplastic cells...
- A new strain of rat for functional analysis of PINASamreen Ahmed
Department of Molecular and Integrative Physiology, University of Michigan Medical School, 7629 MS II, 1301 East Catherine Street, Ann Arbor, MI 48109 0622, USA
Brain Res Mol Brain Res 137:63-9. 2005Long Evans cinnamon (LEC) rat is an animal model for human Wilson disease (WD) due to a deletion in Atp7b, the copper transporter defective in WD patients...
- Expression of copper-transporting P-type adenosine triphosphatase (ATP7B) as a prognostic factor in human endometrial carcinomaTakeshi Aida
Department of Surgery II, Fukushima Medical University, Fukushima, Hikarigaoka 1, 960 1295, Japan
Gynecol Oncol 97:41-5. 2005Copper-transporting P-type adenosine triphosphate (ATP7B) has been reported to be associated with cisplatin resistance in vitro...
- Identification of the "missing domain" of the rat copper-transporting ATPase, atp7b: insight into the structural and metal binding characteristics of its N-terminal copper-binding domainMike J Tsay
Department of Structural Biology and Biochemistry, The Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada
Biochim Biophys Acta 1688:78-85. 2004Wilson disease is an autosomal disorder of copper transport caused by mutations in the ATP7B gene encoding a copper-transporting P-type ATPase. The Long Evans Cinnamon (LEC) rat is an established animal model for Wilson disease...
- Marginal maternal Zn intake in rats alters mammary gland Cu transporter levels and milk Cu concentration and affects neonatal Cu metabolismShannon L Kelleher
Department of Nutrition, University of California Davis, 95616, USA
J Nutr 133:2141-8. 2003..Rats fed ZD had high mammary gland Ctr1, Atp7A and Atp7B levels, milk Cp activity and Cu concentration...
- Copper-transporting P-type adenosine triphosphatase (ATP7B) as a cisplatin based chemoresistance marker in ovarian carcinoma: comparative analysis with expression of MDR1, MRP1, MRP2, LRP and BCRPKentaro Nakayama
Department of Pathology Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan
Int J Cancer 101:488-95. 2002..Recently, copper-transporting P-type adenosine triphosphatase (ATP7B) has been demonstrated as one of the genes responsible for cisplatin resistance in vitro...