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Gene Review

Hbb-bh1  -  hemoglobin Z, beta-like embryonic chain

Mus musculus

Synonyms: Beta-H1-globin, Hemoglobin beta-H1 chain, Hemoglobin subunit beta-H1, Protein Z, beta Hl globin, ...
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Disease relevance of Hbb-bh1

  • Two RING finger proteins, the oncoprotein PML and the arenavirus Z protein, colocalize with the nuclear fraction of the ribosomal P proteins [1].
  • A similar result was observed with a Z protein from another arenavirus, Lassa virus, suggesting that this is a general feature of the Arenaviridae [2].
  • Here we propose that PML reduces LCMV transcription through its interaction with the Z protein [3].
  • We previously showed that hybrid gene constructs of the human fetal G gamma- or mouse embryonic beta h1-globin promoter linked to the viral simian virus 40 T antigen (G gamma/T and beta h1/T) expressed appropriately in embryonic erythroid tissue, with some unexpected expression elsewhere [4].
  • Alteration of additional factors other than accumulation of Z protein, for example inactivation of the endogenous mouse genes or some environmental challenge, might produce a mouse model with more relevance to neonatal liver disease [5].

High impact information on Hbb-bh1

  • However, in this study, we demonstrate that deletion of murine 5' HS3 reduces expression of the linked embryonic epsilon y- and beta H 1-globin genes only minimally in yolk sac-derived erythroid cells and reduces output of the linked adult beta (beta major plus beta minor) globin genes by approximately 30% in adult erythrocytes [6].
  • No benzidine-positive cells or beta H1-globin mRNA expression was detected at the primitive streak or neural plate stage of development (E7.5) [7].
  • Z RNA contains the same open reading frame as C4 which predicts a protein of 131 amino acids, but antisera to C4 do not interact with epitopes on this protein when it is synthesized by cell-free translation, hence the presence or absence of a Z protein in vivo could not be determined [8].
  • Liver injury is caused by the accumulation of alpha(1)-AT mutant Z protein in hepatocytes, which triggers downstream intracellular injury pathways [9].
  • The Z protein is a small zinc-binding RING protein with an unknown function which is required for the viral life cycle [10].

Biological context of Hbb-bh1


Anatomical context of Hbb-bh1


Associations of Hbb-bh1 with chemical compounds

  • Modulation of hepatic heme-binding Z protein in mice by the porphyrogenic carcinogens griseofulvin and hexachlorobenzene [12].

Analytical, diagnostic and therapeutic context of Hbb-bh1


  1. Two RING finger proteins, the oncoprotein PML and the arenavirus Z protein, colocalize with the nuclear fraction of the ribosomal P proteins. Borden, K.L., Campbelldwyer, E.J., Carlile, G.W., Djavani, M., Salvato, M.S. J. Virol. (1998) [Pubmed]
  2. An arenavirus RING (zinc-binding) protein binds the oncoprotein promyelocyte leukemia protein (PML) and relocates PML nuclear bodies to the cytoplasm. Borden, K.L., Campbell Dwyer, E.J., Salvato, M.S. J. Virol. (1998) [Pubmed]
  3. Role of the promyelocytic leukemia protein PML in the interferon sensitivity of lymphocytic choriomeningitis virus. Djavani, M., Rodas, J., Lukashevich, I.S., Horejsh, D., Pandolfi, P.P., Borden, K.L., Salvato, M.S. J. Virol. (2001) [Pubmed]
  4. Prostate, adrenocortical, and brown adipose tumors in fetal globin/T antigen transgenic mice. Perez-Stable, C., Altman, N.H., Brown, J., Harbison, M., Cray, C., Roos, B.A. Lab. Invest. (1996) [Pubmed]
  5. Evaluation of a transgenic mouse model for alpha-1-antitrypsin (AAT) related liver disease. Ali, R., Perfumo, S., della Rocca, C., Amicone, L., Pozzi, L., McCullagh, P., Millward-Sadler, H., Edwards, Y., Povey, S., Tripodi, M. Ann. Hum. Genet. (1994) [Pubmed]
  6. Analysis of mice containing a targeted deletion of beta-globin locus control region 5' hypersensitive site 3. Hug, B.A., Wesselschmidt, R.L., Fiering, S., Bender, M.A., Epner, E., Groudine, M., Ley, T.J. Mol. Cell. Biol. (1996) [Pubmed]
  7. Initiation of hematopoiesis and vasculogenesis in murine yolk sac explants. Palis, J., McGrath, K.E., Kingsley, P.D. Blood (1995) [Pubmed]
  8. A promoter within intron 35 of the human C4A gene initiates abundant adrenal-specific transcription of a 1 kb RNA: location of a cryptic CYP21 promoter element? Tee, M.K., Babalola, G.O., Aza-Blanc, P., Speek, M., Gitelman, S.E., Miller, W.L. Hum. Mol. Genet. (1995) [Pubmed]
  9. Indomethacin increases liver damage in a murine model of liver injury from alpha-1-antitrypsin deficiency. Rudnick, D.A., Shikapwashya, O., Blomenkamp, K., Teckman, J.H. Hepatology (2006) [Pubmed]
  10. The lymphocytic choriomeningitis virus RING protein Z associates with eukaryotic initiation factor 4E and selectively represses translation in a RING-dependent manner. Campbell Dwyer, E.J., Lai, H., MacDonald, R.C., Salvato, M.S., Borden, K.L. J. Virol. (2000) [Pubmed]
  11. The effect of proteolytic enzymes on hair follicles of transgenic mice expressing the lac Z-protein in cells of the bulge region. Protopapa, E.E., Gaissert, H., Xenakis, A., Avramiotis, S., Stavrianeas, N., Sekeris, C.E., Schenkel, J., Alonso, A. Journal of the European Academy of Dermatology and Venereology : JEADV. (1999) [Pubmed]
  12. Modulation of hepatic heme-binding Z protein in mice by the porphyrogenic carcinogens griseofulvin and hexachlorobenzene. Vincent, S.H., Smith, A.G., Muller-Eberhard, U. Cancer Lett. (1989) [Pubmed]
  13. A shortened life span of EKLF-/- adult erythrocytes, due to a deficiency of beta-globin chains, is ameliorated by human gamma-globin chains. Lim, S.K., Bieker, J.J., Lin, C.S., Costantini, F. Blood (1997) [Pubmed]
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