Atp7b

Summary

Gene Symbol: Atp7b
Description: ATPase, Cu++ transporting, beta polypeptide
Alias: Hts, PINA, ATPase, Cu++ transporting, beta polypeptide (same as Wilson disease), PINA gene, promoter, copper pump 2, copper-transporting ATPase 2, pineal night-specific ATPase, wilson disease-associated protein homolog
Species: rat

Top Publications

  1. ncbi A novel pineal night-specific ATPase encoded by the Wilson disease gene
    J Borjigin
    Department of Neuroscience, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    J Neurosci 19:1018-26. 1999
  2. ncbi Liver ischemia and ischemia-reperfusion induces and trafficks the multi-specific metal transporter Atp7b to bile duct canaliculi: possible preferential transport of iron into bile
    John 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. 2008
  3. ncbi Effects of copper supplementation on copper absorption, tissue distribution, and copper transporter expression in an infant rat model
    Kathryn 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
  4. pmc Clusterin and COMMD1 independently regulate degradation of the mammalian copper ATPases ATP7A and ATP7B
    Stephanie 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. 2012
  5. ncbi Mammary gland copper transport is stimulated by prolactin through alterations in Ctr1 and Atp7A localization
    Shannon L Kelleher
    Department of Nutrition, University of California Davis, Davis, CA 95616, USA
    Am J Physiol Regul Integr Comp Physiol 291:R1181-91. 2006
  6. ncbi Dominant-stable beta-catenin expression causes cell fate alterations and Wnt signaling antagonist expression in a murine granulosa cell tumor model
    Derek Boerboom
    Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, Texas 77030, USA
    Cancer Res 66:1964-73. 2006
  7. pmc Aldo-keto reductase 1 family B7 is the gene induced in response to oxidative stress in the livers of Long-Evans Cinnamon rats
    Guang Jia
    Health Effects Research Team, National Institute for Environmental Studies, Tsukuba, Ibaraki 305 8506, Japan
    Int J Oncol 29:829-38. 2006
  8. ncbi Abnormal hepatobiliary and circulating lipid metabolism in the Long-Evans Cinnamon rat model of Wilson's disease
    Emile Levy
    Department of Nutrition, CHU Sainte Justine, Universite de Montreal, 3175 Cote Sainte Catherine, Montreal, Quebec, Canada
    Life Sci 80:1472-83. 2007
  9. doi ATP7B copper-regulated traffic and association with the tight junctions: copper excretion into the bile
    Sonia 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. 2008
  10. pmc Wilson protein expression, copper excretion and sweat production in sweat glands of Wilson disease patients and controls
    Mark Schaefer
    Department of Gastroenterology and Infections Diseases, University of Heidelberg Medical School, INF 410, D 69120 Heidelberg, Germany
    BMC Gastroenterol 8:29. 2008

Scientific Experts

  • Shannon L Kelleher
  • Yuji Takebayashi
  • E Levy
  • Mark Schaefer
  • Atsuko Kanzaki
  • Kentaro Nakayama
  • Stephanie Materia
  • Julian F B Mercer
  • Virginia Ip
  • John A Goss
  • Sonia Hernandez
  • Derek Boerboom
  • Guang Jia
  • Takeshi Aida
  • Kathryn A Bauerly
  • Y Guo
  • Samreen Ahmed
  • Mike J Tsay
  • Kohji Miyazaki
  • Kunihiko Terada
  • Toshihiro Sugiyama
  • J Borjigin
  • X Li
  • Nouri Neamati
  • Kenji Ogawa
  • Michael A Cater
  • Sharon La Fontaine
  • Leo W J Klomp
  • Johnson J Liu
  • Mark J McKeage
  • Yo Tsuchiya
  • Doris Cassio
  • Feng Qin Gao
  • Søren Nielsen
  • Samuel Wyllie
  • Saul J Karpen
  • Vassiliki Lalioti
  • Ignacio V Sandoval
  • Josefa P García-Ruiz
  • Neal R Barshes
  • S H Snyder
  • Yoshiaki Tsuji
  • Zhiping Zhang
  • Jose Courty
  • JoAnne S Richards
  • Ryoya Takahashi
  • Sophie Dalle
  • Lisa D White
  • Hideko Sone
  • Nobuo Yaegashi
  • L Nyasae
  • Chikako Okamura
  • Bo Lonnerdal
  • Seiichi Takenoshita
  • Jimo Borjigin
  • Kiyoshi Ito
  • L T Braiterman
  • A L Hubbard
  • Takao Shimizu
  • Masashi Higasimoto
  • Jie Deng
  • Suree Narindrasorasak
  • Negah Fatemi
  • Bibudhendra Sarkar
  • John R Forbes
  • J D Gitlin
  • Masahiro Oubu
  • Hiroko Bando
  • Hitoshi Miyashita
  • Manabu Fukumoto
  • Masato Mutoh
  • Masakazu Toi
  • Shiro Mori
  • B Ovodenko
  • A S Payne
  • M M Wang
  • J Deng
  • J Wu
  • Y Yamaguchi
  • N Sasaki
  • Q Wang
  • D J Zack
  • S Chen
  • T Kuramoto
  • H S Chen
  • T Agui
  • M Muramatsu
  • A Naito
  • M E Heiny
  • Y Matsuda

Detail Information

Publications22

  1. ncbi A novel pineal night-specific ATPase encoded by the Wilson disease gene
    J Borjigin
    Department of Neuroscience, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    J Neurosci 19:1018-26. 1999
    We have identified a pineal night-specific ATPase (PINA), a novel splice variant of the ATP7B gene disrupted in Wilson disease (WD)...
  2. ncbi Liver ischemia and ischemia-reperfusion induces and trafficks the multi-specific metal transporter Atp7b to bile duct canaliculi: possible preferential transport of iron into bile
    John 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. 2008
    Both 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...
  3. ncbi Effects of copper supplementation on copper absorption, tissue distribution, and copper transporter expression in an infant rat model
    Kathryn 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...
  4. pmc Clusterin and COMMD1 independently regulate degradation of the mammalian copper ATPases ATP7A and ATP7B
    Stephanie 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. 2012
    ATP7A and ATP7B are copper-transporting P(1B)-type ATPases (Cu-ATPases) that are critical for regulating intracellular copper homeostasis...
  5. ncbi Mammary gland copper transport is stimulated by prolactin through alterations in Ctr1 and Atp7A localization
    Shannon 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 ..
  6. ncbi Dominant-stable beta-catenin expression causes cell fate alterations and Wnt signaling antagonist expression in a murine granulosa cell tumor model
    Derek 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...
  7. pmc Aldo-keto reductase 1 family B7 is the gene induced in response to oxidative stress in the livers of Long-Evans Cinnamon rats
    Guang Jia
    Health Effects Research Team, National Institute for Environmental Studies, Tsukuba, Ibaraki 305 8506, Japan
    Int J Oncol 29:829-38. 2006
    The 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...
  8. ncbi Abnormal hepatobiliary and circulating lipid metabolism in the Long-Evans Cinnamon rat model of Wilson's disease
    Emile Levy
    Department of Nutrition, CHU Sainte Justine, Universite de Montreal, 3175 Cote Sainte Catherine, Montreal, Quebec, Canada
    Life Sci 80:1472-83. 2007
    Long-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...
  9. doi ATP7B copper-regulated traffic and association with the tight junctions: copper excretion into the bile
    Sonia 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. 2008
    The 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...
  10. pmc Wilson protein expression, copper excretion and sweat production in sweat glands of Wilson disease patients and controls
    Mark 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...
  11. pmc Differential expression of ATP7A, ATP7B and CTR1 in adult rat dorsal root ganglion tissue
    Virginia 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. 2010
    ATP7A, 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 ..
  12. ncbi NH2-terminal signals in ATP7B Cu-ATPase mediate its Cu-dependent anterograde traffic in polarized hepatic cells
    Y Guo
    Department of Cell Biology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    Am J Physiol Gastrointest Liver Physiol 289:G904-16. 2005
    Cu 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 ..
  13. ncbi A new strain of rat for functional analysis of PINA
    Samreen 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. 2005
    Long 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...
  14. ncbi Expression of copper-transporting P-type adenosine triphosphatase (ATP7B) as a prognostic factor in human endometrial carcinoma
    Takeshi Aida
    Department of Surgery II, Fukushima Medical University, Fukushima, Hikarigaoka 1, 960 1295, Japan
    Gynecol Oncol 97:41-5. 2005
    Copper-transporting P-type adenosine triphosphate (ATP7B) has been reported to be associated with cisplatin resistance in vitro...
  15. pmc Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat
    Y 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...
  16. ncbi The gene responsible for LEC hepatitis, located on rat chromosome 16, is the homolog to the human Wilson disease gene
    N Sasaki
    RIKEN Tsukuba Life Science Center, Institute of Physical and Chemical Research, Ibaraki, Japan
    Biochem Biophys Res Commun 202:512-8. 1994
    We 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...
  17. pmc A pineal regulatory element (PIRE) mediates transactivation by the pineal/retina-specific transcription factor CRX
    X 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...
  18. ncbi Copper-transporting P-type adenosine triphosphatase (ATP7B) is expressed in human breast carcinoma
    Atsuko 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. 2002
    This 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...
  19. ncbi 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 BCRP
    Kentaro 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...
  20. ncbi Marginal maternal Zn intake in rats alters mammary gland Cu transporter levels and milk Cu concentration and affects neonatal Cu metabolism
    Shannon 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...
  21. ncbi 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 domain
    Mike J Tsay
    Department of Structural Biology and Biochemistry, The Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada
    Biochim Biophys Acta 1688:78-85. 2004
    Wilson 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...
  22. ncbi The LEC rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease gene
    J 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 ..