| 1. |
- Hamaguchi, Isao, et al.
(författare)
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Proliferation deficiency of multipotent hematopoietic progenitors in ribosomal protein S19 (RPS19)-deficient Diamond-Blackfan anemia improves following RPS19 gene transfer
- 2003
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Ingår i: Molecular Therapy. - 1525-0024. ; 7:5, s. 613-622
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Tidskriftsartikel (refereegranskat)abstract
- Diamond-Blackfan anemia (DBA) is a congenital bone marrow failure syndrome characterized by a specific deficiency in erythroid progenitors. Since some patients with DBA develop a reduction in thrombocytes and granulocytes with age, we asked whether multipotent hematopoietic progenitors from DBA patients had normal proliferative capacity in liquid expansion cultures. CD34(+) cells derived from DBA patients showed deficient proliferation in liquid culture containing IL-3, IL-6, and SCF. Single CD34(+) CD38(-) cells from DBA patients exhibited deficient proliferation recruitment in a limiting dilution assay containing IL-3, IL-6, SCF, Tpo, FIL, and G-CSF or containing IL-3, IL-6, and SCF. Our findings suggest that the underlying hematopoietic defect in DBA may not be limited to the erythroid lineage. Since a fraction of DBA patients have a deficiency in ribosomal protein S19 (RPS19), we constructed lentiviral vectors containing the RPS19 gene for overexpression in hematopoietic progenitors from RPS19-deficient DBA patients. Enforced expression of the RPS19 transgene improved the proliferation of CD34(+) cells from DBA patients with RPS19 mutation. Similarly, enforced expression of RPS19 improved erythroid development of RPS19-deficient hematopoietic progenitors as determined by colony assays and erythroid differentiation cultures. These findings suggest that gene therapy for RPS19-deficient DBA is feasible.
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| 2. |
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| 3. |
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| 4. |
- Ahrens, J., et al.
(författare)
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Results from AMANDA
- 2001
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Ingår i: Proceedings, 9th International Workshop, Venice, Italy, March 6-9, 2001. Vol. 1, 2. ; , s. 569-580
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Konferensbidrag (refereegranskat)
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| 5. |
- Ekberg, Jenny, et al.
(författare)
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Regulation of the cyclin A1 protein is associated with its differential subcellular localization in hematopoietic and leukemic cells
- 2004
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Ingår i: Oncogene. - : Nature Publishing Group. - 0950-9232 .- 1476-5594. ; 23:56, s. 9082-9089
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Tidskriftsartikel (refereegranskat)abstract
- An important role of the cell cycle regulatory protein cyclin A1 in the development of acute myeloid leukemia (AML) was previously demonstrated in a transgenic mouse model. We have now turned our attention to study specific aspects of the activity and subcellular distribution of cyclin A1 using bone marrow samples from normal donors and patients with AML, as well as leukemic cell lines. We show that the localization of cyclin A1 in normal hematopoietic cells is nuclear, whereas in leukemic cells from AML patients and cell lines, it is predominantly cytoplasmic. In leukemic cell lines treated with all-trans retinoic acid (ATRA), cyclin A1 localized to the nucleus. Further, there was a direct interaction between cyclin A1 and cyclin-dependent kinase 1, as well as a major ATRA receptor, RARalpha, in ATRA-treated cells but not in untreated leukemic cells. Our results indicate that the altered intracellular distribution of cyclin A1 in leukemic cells correlates with the status of the leukemic phenotype.
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| 6. |
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| 7. |
- Hamaguchi, Isao, et al.
(författare)
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Gene transfer improves erythroid development in ribosomal protein S19-deficient Diamond-Blackfan anemia.
- 2002
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Ingår i: Blood. - 1528-0020. ; 100:8, s. 2724-2731
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Tidskriftsartikel (refereegranskat)abstract
- Diamond-Blackfan anemia (DBA) is a congenital bone marrow failure syndrome characterized by a specific deficiency in erythroid progenitors. Forty percent of the patients are blood transfusion-dependent. Recent reports show that the ribosomal protein S19 (RPS19) gene is mutated in 25% of all patients with DBA. We constructed oncoretroviral vectors containing the RPS19 gene to develop gene therapy for RPS19-deficient DBA. These vectors were used to introduce the RPS19 gene into CD34(+) bone marrow (BM) cells from 4 patients with DBA with RPS19 gene mutations. Overexpression of the RPS19 transgene increased the number of erythroid colonies by almost 3-fold. High expression levels of the RPS19 transgene improved erythroid colony-forming ability substantially whereas low expression levels had no effect. Overexpression of RPS19 had no detrimental effect on granulocyte-macrophage colony formation. Therefore, these findings suggest that gene therapy for RPS19-deficient patients with DBA using viral vectors that express the RPS19 gene is feasible. (Blood. 2002;100:2724-2731)
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| 8. |
- Hardalupas, Y, et al.
(författare)
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Chemiluminescence sensor for local equivalence ratio of reacting mixtures of fuel and air (FLAMESEEK)
- 2004
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Ingår i: Applied Thermal Engineering. - : Elsevier BV. - 1359-4311. ; 24:11-12, s. 1619-1632
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Tidskriftsartikel (refereegranskat)abstract
- This paper describes a Cassegrain optics-based chemiluminescence sensor (CS) for measurements in gas turbine combustors. The chemiluminescence sensor measures the equivalence ratio of reacting fuel and air mixtures, and can identify the flame location, in partially premixed flames. It has the potential for monitoring the degree of premixedness of reacting fuel and air in industrial gas turbine combustors, where operation with lean premixed mixtures is important for reduction of NO, emissions. The spatial resolution of the sensor is evaluated by comparing OH* chemiluminescence measurement from the CS with laser induced OH fluorescence, in the cone-shaped premixed flame of a Bunsen burner. The ability of the sensor to measure in a modified micro-gas turbine environment burning a methane/air, as well as, a methane/ water/air flame (humidified flame) is also demonstrated.
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| 9. |
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| 10. |
- Hult, Johan, et al.
(författare)
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Application of a high-repetition-rate laser diagnostic system for single-cycle-resolved imaging in internal combustion engines
- 2002
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Ingår i: Applied Optics. - 2155-3165. ; 41:24, s. 5002-5014
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Tidskriftsartikel (refereegranskat)abstract
- High-repetition-rate laser-induced fluorescence measurements of fuel and OH concentrations in internal combustion engines are demonstrated. Series of as many as eight fluorescence images, with a temporal resolution ranging from 10 mus to 1 ms, are acquired within one engine cycle. A multiple-laser system in combination with a multiple-CCD camera is used for cycle-resolved imaging in spark-ignition, direct-injection stratified-charge, and homogeneous-charge compression-ignition engines. The recorded data reveal unique information on cycle-to-cycle variations in fuel transport and combustion. Moreover, the imaging system in combination with a scanning mirror is used to perform instantaneous three-dimensional fuel-concentration measurements.
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