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  • 1
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    Correction of sickle cell disease in transgenic mouse models by gene therapy (2001)
    American Association for the Advancement of Science (AAAS)
    Details
    Publication Date: 2001-12-18
    Description: Sickle cell disease (SCD) is caused by a single point mutation in the human betaA globin gene that results in the formation of an abnormal hemoglobin [HbS (alpha2betaS2)]. We designed a betaA globin gene variant that prevents HbS polymerization and introduced it into a lentiviral vector we optimized for transfer to hematopoietic stem cells and gene expression in the adult red blood cell lineage. Long-term expression (up to 10 months) was achieved, without preselection, in all transplanted mice with erythroid-specific accumulation of the antisickling protein in up to 52% of total hemoglobin and 99% of circulating red blood cells. In two mouse SCD models, Berkeley and SAD, inhibition of red blood cell dehydration and sickling was achieved with correction of hematological parameters, splenomegaly, and prevention of the characteristic urine concentration defect.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Pawliuk, R -- Westerman, K A -- Fabry, M E -- Payen, E -- Tighe, R -- Bouhassira, E E -- Acharya, S A -- Ellis, J -- London, I M -- Eaves, C J -- Humphries, R K -- Beuzard, Y -- Nagel, R L -- Leboulch, P -- HL554352/HL/NHLBI NIH HHS/ -- New York, N.Y. -- Science. 2001 Dec 14;294(5550):2368-71.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Harvard-MIT, Division of Health Sciences and Technology, Massachusetts Institute of Technology, Cambridge, MA 02139, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/11743206" target="_blank"〉PubMed〈/a〉
    Keywords: Anemia, Sickle Cell/genetics/*therapy ; Animals ; Disease Models, Animal ; Erythrocytes/metabolism ; Gene Expression ; *Genetic Therapy ; *Genetic Vectors ; Globins/*genetics/metabolism ; HIV-1/*genetics ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells/metabolism ; Hemoglobin, Sickle/metabolism ; Humans ; Lentivirus/genetics ; Locus Control Region ; Mice ; Mice, Inbred C57BL ; Mice, Transgenic ; Oxyhemoglobins/metabolism ; RNA, Messenger/genetics/metabolism ; Thalassemia/genetics/therapy ; Transduction, Genetic ; Transgenes ; beta-Globins
    Print ISSN: 0036-8075
    Electronic ISSN: 1095-9203
    Topics: Biology , Chemistry and Pharmacology , Computer Science , Medicine , Natural Sciences in General , Physics
    Published by American Association for the Advancement of Science (AAAS)
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  • 2
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    Malaria red cell cytoadherence (1989)
    American Association for the Advancement of Science (AAAS)
    Details
    Publication Date: 1989-11-24
    Description: 〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Roth, E Jr -- Kaul, D K -- Nagel, R L -- New York, N.Y. -- Science. 1989 Nov 24;246(4933):1051.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/2686026" target="_blank"〉PubMed〈/a〉
    Keywords: Animals ; Cell Adhesion ; Erythrocytes/cytology/*parasitology ; Humans ; Malaria/*blood ; Plasmodium falciparum/pathogenicity
    Print ISSN: 0036-8075
    Electronic ISSN: 1095-9203
    Topics: Biology , Chemistry and Pharmacology , Computer Science , Medicine , Natural Sciences in General , Physics
    Published by American Association for the Advancement of Science (AAAS)
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  • 3
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    Sickling rates of human AS red cells infected in vitro with Plasmodium falciparum malaria (1978)
    American Association for the Advancement of Science (AAAS)
    Details
    Publication Date: 1978-11-10
    Description: The kinetics of sickling of malaria-infected red cells from humans with sickle cell trait were studied in vitro in an attempt to obtain direct experimental evidence for a selective advantage of the hemoglobin S heterozygote in a malarious region. The sickling rates of cells infected with Plasmodium falciparum and of non-infected cells were studied both in the total absence of oxygen (by dithionite addition) and at several different concentrations of oxyhemoglobin which might obtain in vivo. In all cases, red cells containing small plasmodium parasite forms (ring forms) sickled approximately eight times as readily as uninfected cells. Cells containing large parasitic forms (trophozoites and schizonts) appeared to sickle less readily than uninfected cells, by light microscopy criteria, but electron micrographs demonstrated the presence of polymerized deoxyhemoglobin S with a high frequency. It is concluded that enhanced sickling of plasmodium-infected AS cells may be one mechanism whereby the hemoglobin S polymorphism is balanced in favor of the heterozygote.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Roth, E F Jr -- Friedman, M -- Ueda, Y -- Tellez, I -- Trager, W -- Nagel, R L -- New York, N.Y. -- Science. 1978 Nov 10;202(4368):650-2.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/360396" target="_blank"〉PubMed〈/a〉
    Keywords: Anemia, Sickle Cell/*parasitology ; Erythrocytes, Abnormal/*parasitology ; Heterozygote ; Humans ; Kinetics ; Malaria/*blood ; Plasmodium falciparum ; Sickle Cell Trait/parasitology
    Print ISSN: 0036-8075
    Electronic ISSN: 1095-9203
    Topics: Biology , Chemistry and Pharmacology , Computer Science , Medicine , Natural Sciences in General , Physics
    Published by American Association for the Advancement of Science (AAAS)
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  • 4
    Electronic Resource
    Electronic Resource
    Rate of activation and deactivation of K∶Cl cotransport by changes in cell volume in hemoglobin SS, CC and AA red cells (1994)
    Springer
    The journal of membrane biology 142 (1994), S. 349-362 
    Details
    ISSN: 1432-1424
    Keywords: Hemoglobin S ; Hemoglobin C ; Red blood cells ; KCl cotransport ; Sickle cell anemia ; Cell volume regulation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology
    Notes: Abstract Red blood cells (RBC) of subjects homozygous for hemoglobin A (AA), C (CC) and S (SS) exhibit different cell volumes which might be related to differences in cell volume regulation. We have investigated how rapidly K:Cl cotransport is activated and deactivated to regulate the cell volume in these cells. We measured the time course of net K+ efflux after step changes in cell volume and determined two delay times: one for activation by cell swelling and a second for deactivation by cell shrinkage. Cell swelling induced by 220 mOsm media activated K+ efflux to high values (10–20 mmol/ liter cell x hr) in CC and SS; normal AA had a threefold lower activity. The delay time for activation was very short in blood with a high percentage of reticulocytes (retics): (SS, 10% retics, 1.7±0.3 min delay, n=8; AA, 10% retics, 4±1.5 min, n=3; CC, 11.6% retics, 4±0.3, n=3) and long in cells with a smaller percentage of reticulocytes: (AA, 1.5% retics, 10±1.4 min, n=8; CC whole blood 6% retics, 10±2.0 min, n=10, P〈0.02 vs. SS). The delay times for deactivation by cell shrinking were very short in SS (3.6±0.4 min, n=8, P〈0.02) and AA cells with high retics (2.7±1 min, n=3) and normal retics (2.8±1 min, n=3), but 8–15-fold longer in CC cells (29±2.8 min, n=9). Density fractionation of CC cells (n=3) resulted in coenrichment of the top fraction in reticulocytes and in swelling-activated cotransport (fourfold) with short delay time for activation (4±0.3 min) and long delay for deactivation (14±4 min). The delay time for activation, but not for deactivation, increased markedly with increasing cell density. These findings indicate that all CC cells do not promptly shut off cotransport with cell shrinkage and high rates of cellular K+ loss persist after return to isotonic conditions. In summary, (i) K:Cl cotransport is not only very active in young cells but it is also very rapidly activated and deactivated in young AA and SS cells by changes in cell volume. (ii) Delay times for cotransport activation markedly increased with RBC age and in mature cells with low cotransport rates, long delay times for activation were observed. (iii) The long delay time for deactivation exhibited even by young CC cells induces a persistent loss of K+ after cell shrinkage which may contribute in vivo to the uniformly low cell volume, low K+ and water content of CC cells.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00233441
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  • 5
    Electronic Resource
    Electronic Resource
    Experience with an Animal Model for Sickle Cell Vasoocclusion (1989)
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 565 (1989), S. 0 
    Details
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1749-6632.1989.tb24195.x
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  • 6
    Electronic Resource
    Electronic Resource
    Erythrocytic and Vascular Factors Influencing the Microcirculatory Behavior of Blood in Sickle Cell Anemia (1989)
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 565 (1989), S. 0 
    Details
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1749-6632.1989.tb24179.x
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  • 7
    Electronic Resource
    Electronic Resource
    Relation of the Peripheral Percent Fetal Hemoglobin Level and Burst-Promoting Activity Production to the Regulation of the Circulating Erythroid Progenitor Cell Population in Sickle Cell Anemia (1989)
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 565 (1989), S. 0 
    Details
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1749-6632.1989.tb24193.x
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  • 8
    Electronic Resource
    Electronic Resource
    Hemoglobins S and C in Upper Volta (1984)
    Springer
    Human genetics 〈Berlin〉 65 (1984), S. 300-302 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary We have studied the incidence of hemoglobinopathies in 1059 individuals in Upper Volta. We have found that this population has a high frequency of HbS and HbC and α-thalassemia. The gene frequency of HbS was high (0.1 for the βS gene) in the arid Sahel portion of Upper Volta accompanied by a lower frequency for HbC (0.05 for the βc gene). The reverse was true in the humid Savanna region of this country (0.03 for the βS gene and 0.14 for the βc gene). There was no age dependency of the HbS gene frequency, but α-thalassemia, detectable in HbS heterozygotes, showed a statistically significant decrease with age. No homozygote for HbS was detected after the age of 1 year, and SC and CC genotypes were found at a lower incidence than expected. The environmental and medical conditions in Upper Volta preclude the survival of SS individuals and decrease the survival of SC and CC genotypes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00286522
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  • 9
    Electronic Resource
    Electronic Resource
    β -Chain contact sites in the haemoglobin S polymer (1980)
    [s.l.] : Nature Publishing Group
    Nature 283 (1980), S. 832-834 
    Details
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] The amino acid residues involved in the areas of contact that stabilise the haemoglobin S polymer fibre seem to be the same ones that stabilise the basic unit of the deoxyhaemoglobin S crystal: the Wishner–Love double strand. The haemoglobin S fibre is probably formed by a unique packing of ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/283832a0
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  • 10
    Electronic Resource
    Electronic Resource
    α-Thalassemia among sickle cell anemia patients in various African populations (1984)
    Springer
    Human genetics 〈Berlin〉 68 (1984), S. 318-319 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary We have studied the incidence of α-thalassemia in normal and SS individuals from Senegal, Benin, Upper Volta, and Central Republican Africa. The α thal gene frequency is not significantly different in the controls from the various populations and in the SS patients from Senegal. In contrast it is compatible with increased survival of SS patients in Benin, Upper Volta. The data suggest epistatic effects of other factors in the Senegalese population.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00292592
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