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Gene Review

DBA2  -  Diamond-Blackfan anemia 2

Homo sapiens

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Disease relevance of DBA2

  • When DBA2 mice were immunized and boosted with 200 pfu of a LCMV mutant, the neutralizing Ab response was limited to the immunizing "personal" clone [1].
  • Hematopoietic transplant performed after onset of disease resulted in 42% mortality in SJL/J syngeneic transplants, 47% mortality in diseased DBA2 recipients restored with marrow from naive B6D2 donors, and 12% in diseased DBA2 recipients receiving marrow from B6D2 donors previously infected with TMEV [2].
  • Tamoxifen (TX) and toremifene (TO) enhanced the lysis of P815 mastocytoma cells in vitro by syngeneic DBA2 spleen cells that have been activated by human recombinant interleukin-2 (IL-2) for 6 days (lymphokine-activated killer [LAK] cells) [3].
  • Five different multidrug-resistant cell lines, characterized by mdr1 amplification, were used in this study: Dox-R-MCF7, a human breast adenocarcinoma; SKVBL1, a human ovarian adenocarcinoma; K562-R, a human erythroleukemia; and two murine lines: P388-Adr-R, a monocytic leukemia of DBA2 mouse, and LR73MDR, a Chinese hamster ovarian cell line [4].
  • Both compounds were able to target 111In to Cloudman S91 melanomas in DBA2 mice [5].

High impact information on DBA2

  • However, when TCDD was administered, a greatly diminished response was observed in homozygous hAHR mice compared with Ahr(d/d) mice, indicating that hAHR expressed in mice is functionally less responsive to TCDD than DBA2 AHR [6].
  • Responses of these mice to 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) and 3-methylcholanthrene were compared with the responses of naturally sensitive (C57BL6J) and resistant (DBA2) mice [6].
  • In most inbred DBA2 mice infected with 2 x 10(4) or 2 x 10(6) plaque-forming units (pfu) of lymphocytic choriomeningitis virus (LCMV), the virus is transiently controlled below detectable levels measured with conventional assays (<1.7 pfu), but reemerges despite a common neutralizing Ab (nAb) response [1].
  • In contrast, at the proximal tibia, C3H/HeJ mice had a greater decline in trabecular BV/TV (-39%) than C57BL/6J (-18%), DBA2/J (-23%), and CAST/EiJ mice (-21%) [7].
  • Similarly, enhanced tumor suppression occurred when TX- or TO-treated P815 cells were mixed with LAK cells and injected s.c. into normal DBA2 recipients [3].

Biological context of DBA2


Anatomical context of DBA2

  • The treatment-induced changes in circulating testosterone in NZB dams and C57BL/6 dams were not reflected in serum from 18-day NZB/W or C57/DBA2 fetuses [13].
  • We have compared the inhibitory effect of rubidazone to that of adriamycin on P388 leukaemia and normal bone marrow colony-forming units (CFU) using the spleen colony assay system in male DBA2 mice [14].
  • These also reacted with the murine equivalent of the human R80K and were used in inhibition studies of natural killer (NK) cell killing and the mouse abortion models, CBA x DBA2 F1 resorption in CBA females, the endotoxin-induced resorption model, and a sonic stress-induced murine resorption model [15].
  • Mixture of blood lymphocytes, phytohemagglutinin and Cr51 labeled target cells (P815/DBA2) were incubated in ratio 10:1 for 12 hours [16].
  • Strain specific variation in the immediate survival of donor male myoblasts following MTT in untreated C57BL/10Sn, DBA-1 and DBA-2 (C5-deficient) female hosts was observed [12].

Associations of DBA2 with chemical compounds

  • Our findings suggest that placental androgen control is regulated differently in the autoimmune NZB-NZB/W vs. the nonautoimmune C57BL/6-C57/DBA2 maternal-placental-fetal unit [13].
  • The results of this study show that the level of CHP, a peptide that mimics dopamine in many of its pharmacologic actions, is lower in brains of alcohol-preferring C57BL mice compared to alcohol non-preferring DBA2 mice [17].
  • Cobra venom factor depletion of C3 increased initial donor male myoblast survival (approximately twofold at 0 h) in C57BL/10Sn and DBA-1 host mice and approximately threefold in DBA-2 hosts at 0 h and 1 h after MTT [12].
  • In rodents, it has a wide spectrum of activity preventing seizures induced by picrotoxin, pentylenetetrazol, i.c.v. 4-aminopyridine or NMDA, and audiogenic seizures in DBA-2 mice and GEPR-9 rats [18].

Analytical, diagnostic and therapeutic context of DBA2

  • MATERIALS AND METHODS: Four-month-old mice from five inbred mouse strains (C3H/HeJ, BALB/cByJ, CAST/EiJ, DBA2/J, and C57BL/6J) underwent ovariectomy (OVX) or sham-OVX surgery (n = 6-9/group) [7].
  • Intraperitoneal ubiquitin administration (25 mug/h for 14 days) was well-tolerated, dose-dependently increased ubiquitin serum concentrations and median allograft survival from 10 days (with albumin; control) to 17 days in DBA2 mice (survival ratio: 1.7, 95% confidence interval: 1.266-2.134; P=0.0005) [19].
  • Eight-week-old male DBA-2 mice were infected with EMC virus and randomized to a treatment or control group [20].
  • The ability of iron to stimulate the growth of L1210 cells both in DBA-2 mice and in cell culture is evaluated [21].


  1. Public versus personal serotypes of a viral quasispecies. Hunziker, L., Ciurea, A., Recher, M., Hengartner, H., Zinkernagel, R.M. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  2. Viral hyperinfection of the central nervous system and high mortality after hematopoietic stem cell transplantation for treatment of Theiler's murine encephalomyelitis virus-induced demyelinating disease. Burt, R.K., Padilla, J., Dal Canto, M.C., Miller, S.D. Blood (1999) [Pubmed]
  3. Anti-estrogens enhance the therapeutic effect of lymphokine-activated killer cells on the P815 murine mastocytoma. Baral, E., Nagy, E., Kangas, L., Berczi, I. Int. J. Cancer (1996) [Pubmed]
  4. Doxorubicin-loaded nanospheres bypass tumor cell multidrug resistance. Cuvier, C., Roblot-Treupel, L., Millot, J.M., Lizard, G., Chevillard, S., Manfait, M., Couvreur, P., Poupon, M.F. Biochem. Pharmacol. (1992) [Pubmed]
  5. An improved imaging agent for malignant melanoma, based on [Nle4,D-Phe7]alpha-melanocyte stimulating hormone. Bard, D.R. Nuclear medicine communications. (1995) [Pubmed]
  6. Distinct response to dioxin in an arylhydrocarbon receptor (AHR)-humanized mouse. Moriguchi, T., Motohashi, H., Hosoya, T., Nakajima, O., Takahashi, S., Ohsako, S., Aoki, Y., Nishimura, N., Tohyama, C., Fujii-Kuriyama, Y., Yamamoto, M. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  7. Ovariectomy-induced bone loss varies among inbred strains of mice. Bouxsein, M.L., Myers, K.S., Shultz, K.L., Donahue, L.R., Rosen, C.J., Beamer, W.G. J. Bone Miner. Res. (2005) [Pubmed]
  8. Strain-specific induction of murine lung tumors following in utero exposure to 3-methylcholanthrene. Jennings-Gee, J.E., Moore, J.E., Xu, M., Dance, S.T., Kock, N.D., McCoy, T.P., Carr, J.J., Miller, M.S. Mol. Carcinog. (2006) [Pubmed]
  9. N-(2-hydroxypropyl)methacrylamide copolymers targeted to the hepatocyte galactose-receptor: pharmacokinetics in DBA2 mice. Seymour, L.W., Ulbrich, K., Wedge, S.R., Hume, I.C., Strohalm, J., Duncan, R. Br. J. Cancer (1991) [Pubmed]
  10. Bilateral microtia and cleft palate in cousins with Diamond-Blackfan anemia. Gripp, K.W., McDonald-McGinn, D.M., La Rossa, D., McGain, D., Federman, N., Vlachos, A., Glader, B.E., McKenzie, S.E., Lipton, J.M., Zackai, E.H. Am. J. Med. Genet. (2001) [Pubmed]
  11. Potentiation by caffeine of the frequencies of micronuclei induced by mitomycin C and cyclophosphamide in young mice. Blagoeva, P.M., Balansky, R.M., Mircheva, T.J. Mutat. Res. (1991) [Pubmed]
  12. Complement and myoblast transfer therapy: donor myoblast survival is enhanced following depletion of host complement C3 using cobra venom factor, but not in the absence of C5. Hodgetts, S.I., Grounds, M.D. Immunol. Cell Biol. (2001) [Pubmed]
  13. Aberrant hormone balance in fetal autoimmune NZB/W mice following prenatal exposure to testosterone excess or the androgen blocker flutamide. Keisler, L.W., vom Saal, F.S., Keisler, D.H., Rudeen, P.K., Walker, S.E. Biol. Reprod. (1995) [Pubmed]
  14. Rubidazone vs adriamycin: an evaluation of their differential toxicity in the spleen colony assay system. Alberts, D.S., Van Daalen Wetters, T. Br. J. Cancer (1976) [Pubmed]
  15. Maternal response to paternal trophoblast antigens. Mowbray, J., Jalali, R., Chaouat, G., Clark, D.A., Underwood, J., Allen, W.R., Mathias, S. Am. J. Reprod. Immunol. (1997) [Pubmed]
  16. The influence of corticotherapy on mitogen induced lymphocytotoxicity in bronchial asthma. Podleski, W.K., Takahashi, M. Annals of allergy. (1978) [Pubmed]
  17. Role of endogenous cyclo(His-Pro) in voluntary alcohol consumption by alcohol-preferring C57BL mice. Prasad, C. Peptides (2001) [Pubmed]
  18. BTS 72664-- a novel CNS drug with potential anticonvulsant, neuroprotective, and antimigraine properties. Smith, S.L., Thompson, K.S., Sargent, B.J., Heal, D.J. CNS drug reviews. (2001) [Pubmed]
  19. Prolongation of skin graft survival by exogenous ubiquitin. Earle, S.A., El-Haddad, A., Patel, M.B., Ruiz, P., Pham, S.M., Majetschak, M. Transplantation (2006) [Pubmed]
  20. Therapy with cyclosporine in experimental murine myocarditis with encephalomyocarditis virus. Monrad, E.S., Matsumori, A., Murphy, J.C., Fox, J.G., Crumpacker, C.S., Abelmann, W.H. Circulation (1986) [Pubmed]
  21. Influence of iron on in vivo proliferation and lethality of L1210 cells. Bergeron, R.J., Streiff, R.R., Elliott, G.T. J. Nutr. (1985) [Pubmed]
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