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Gene Review

Atp7b  -  ATPase, Cu++ transporting, beta polypeptide

Rattus norvegicus

Synonyms: Copper pump 2, Copper-transporting ATPase 2, Hts, PINA, Pina, ...
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Disease relevance of Atp7b


High impact information on Atp7b


Chemical compound and disease context of Atp7b

  • While analyzing the melatonin synthetic pathways of Long Evans cinnamon (LEC) rats mutant for PINA, a pineal night-specific ATPase defective in Wilson disease, we discovered that NAT activity and protein levels are greatly reduced in LEC rats, and that the highly conserved histidine 28 is mutated to tyrosine [5].

Biological context of Atp7b

  • We additionally found that hepatic Atp7b gene expression does not change with iron deficiency, suggesting that liver copper excretion is not altered [6].
  • We have developed lacI transgenic Wilson disease (WND-B) rats by mating LEC with Big Blue F344 rats carrying a lambda shuttle vector harboring the lacI gene. lacI mutations of the livers of C-B heterozygous (Atp7b w/m, lacI) and WND-B homozygous (Atp7b m/m, lacI) rats at 6, 24, and 40 weeks of ages were analyzed [7].
  • At 19 days of gestation, hepatic copper concentrations of (LECXF344)F1XLEC and LECX(LECXF344)F1 fetuses were equivalent to those of F344 and LEC fetuses, respectively, irrespective of their Atp7b genotype [8].
  • In vitro, PINA expression in pineal cells can be stimulated by agents activating the cAMP signal transduction pathway [4].
  • Linkage studies confirm that the NAT phenotype is entirely independent of PINA mutation in the pineal gland of LEC rats, and sequence analysis demonstrates that NAT defect is due to a point mutation in NAT coding region [1].

Anatomical context of Atp7b


Associations of Atp7b with chemical compounds

  • Furthermore, they suggested the possibility that it correlates to copper accumulation due to the Atp7b gene mutation, because ionizing radiation-induced hydroxyl radicals might act in concert with copper-induced hydroxyl radicals [10].
  • Role of Atp7b gene in spontaneous and N-diethylnitrosamine-induced carcinogenesis in a new congenic strain, WKAH.C-Atp7b rats [11].
  • This action of CGRP was sensitive to Glib and had only slight sensitivity to tetraethylammonium; this CGRP effect was mimicked by Pina but not by FSK [12].


  1. A new strain of rat for functional analysis of PINA. Ahmed, S., Deng, J., Borjigin, J. Brain Res. Mol. Brain Res. (2005) [Pubmed]
  2. The LEC rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease gene. Wu, J., Forbes, J.R., Chen, H.S., Cox, D.W. Nat. Genet. (1994) [Pubmed]
  3. A pineal regulatory element (PIRE) mediates transactivation by the pineal/retina-specific transcription factor CRX. Li, X., Chen, S., Wang, Q., Zack, D.J., Snyder, S.H., Borjigin, J. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  4. A novel pineal night-specific ATPase encoded by the Wilson disease gene. Borjigin, J., Payne, A.S., Deng, J., Li, X., Wang, M.M., Ovodenko, B., Gitlin, J.D., Snyder, S.H. J. Neurosci. (1999) [Pubmed]
  5. A novel H28Y mutation in LEC rats leads to decreased NAT protein stability in vivo and in vitro. Huang, Z., Deng, J., Borjigin, J. J. Pineal Res. (2005) [Pubmed]
  6. Menkes Copper ATPase (Atp7a) is a novel metal-responsive gene in rat duodenum, and immunoreactive protein is present on brush-border and basolateral membrane domains. Ravia, J.J., Stephen, R.M., Ghishan, F.K., Collins, J.F. J. Biol. Chem. (2005) [Pubmed]
  7. Increased mutant frequency and altered mutation spectrum of the lacI transgene in Wilson disease rats with hepatitis. Sone, H., Li, Y.J., Ishizuka, M., Aoki, Y., Nagao, M. Cancer Res. (2000) [Pubmed]
  8. Fetal copper uptake and a homolog (Atp7b) of the Wilson's disease gene in rats. Muramatsu, Y., Yamada, T., Moralejo, D.H., Suzuki, Y., Matsumoto, K. Res. Commun. Mol. Pathol. Pharmacol. (1998) [Pubmed]
  9. Effects of soy protein isolate on LEC rats, a model of Wilson disease: mechanisms underlying enhancement of liver cell damage. Yonezawa, K., Nakagama, H., Tajima, R., Ushigome, M., Ogra, Y., Suzuki, K.T., Yoshikawa, K., Nagao, M. Biochem. Biophys. Res. Commun. (2003) [Pubmed]
  10. Absence of linkage between radiosensitivity and the predisposing atp7b gene mutation for heritable hepatitis in the LEC rat. Ogiu, T., Nishimura, M., Watanabe, F., Ukai, H., Ishii-Ohba, H., Shimada, Y., Tsuji, H., Sakurai, J., Hino, O. Radiat. Res. (2000) [Pubmed]
  11. Role of Atp7b gene in spontaneous and N-diethylnitrosamine-induced carcinogenesis in a new congenic strain, WKAH.C-Atp7b rats. Minami, T., Kaneda, S., Otsuka, T., Jiao, Z., Suzuki, Y., Yamada, T., Matsumoto, K., Izumi, K. Jpn. J. Cancer Res. (2001) [Pubmed]
  12. Calcitonin gene-related peptide stimulates potassium efflux through adenosine triphosphate-sensitive potassium channels and produces membrane hyperpolarization in osteoblastic UMR106 cells. Kawase, T., Burns, D.M. Endocrinology (1998) [Pubmed]
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