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  • 1
    Electronic Resource
    Electronic Resource
    Exon skipping due to a mutation in a donor splice site in the WT-1 gene is associated with Wilms' tumor and severe genital malformations (1993)
    Springer
    Human genetics 〈Berlin〉 91 (1993), S. 599-604 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract A germline WT-1 point mutation is described in a patient with unilateral Wilms' tumor, nephritis and ambiguous external genitalia. The patient was diagnosed as a possible case of Denys Drash syndrome (DDS). Analysis of the WT-1 exons and intron borders revealed a G to C transversion in the +1 position of the splice donor consensus sequence in intron 6. Two transcripts of abnormal size were identified in tumor RNA. Sequencing of the altered WT-1 mRNA revealed that this point mutation leads to exon-skipping, resulting in transcripts either missing exon 6 or exons 5 and 6. The normally occurring alternative splicing of exon 5 in the WT-1 gene is not affected by this mutation. The reading frame is changed when either both exons 5 and 6 or exon 6 alone are missing and a stop codon follows immediately downstream in exon 7. Most mutations identified in DDS are missense mutations located in the zinc finger (ZF) region (exons 7, 8 and 9) but recently a patient with a germline mutation in exon 6 leading to premature chain termination was described. Therefore the site of the mutation in the WT-1 gene in this patient cannot exclude the possibility that he has DDS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00205087
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  • 2
    Electronic Resource
    Electronic Resource
    Circulating haematopoietic progenitors during treatment of renal anaemia with recombinant human erythropoietin (1994)
    Springer
    European journal of pediatrics 153 (1994), S. 43-48 
    Details
    ISSN: 1432-1076
    Keywords: Chronic renal failure Erythropoietin ; Haematopoietic progenitors ; Haemodialysis Interleukin 3
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The effect of recombinant human erythropoietin (rhEPO) and interleukin 3 (IL3) on circulating haematopoietic progenitors consisting mainly of immature burst-forming-units-erythrocytes (BFU-E), was investigated in ten paediatric patients treated by regular haemodialysis. During a 30-week study rhEPO treatment resulted in a rise of median haemoglobin levels from 6.7 g/dl to 〉10 g/dl in all patients. Before initiating rhEPO treatment the number of circulating BFU-E in chronic renal failure patients responded to grading doses of rhEPO in vitro similar to that in control children; however, the dose-response curves were not predictive for the in vivo response to rhEPO. After an initial rise in five patients BFU-E numbers declined by week 30 of rhEPO treatment. BFU-E numbers decreased to 35% of pretreatment values. The number of granulocyte-macrophage colony forming cells (GM-CFC) also decreased during rhEPO treatment. Addition of IL3 to the culture medium containing saturating concentrations of granulocyte-macrophage colony stimulating factor did not stimulate BFU-E numbers of patients before rhEPO treatment or those of controls. However, 2 weeks after start of rhEPO treatment IL3 increased the growth of patient's BFU-E in vitro to 220% of pretreatment levels, followed by a gradual decrease of stimulation until the end of observation. These findings indicate that: (1) long-term recruitment of circulating haematopoietic progenitors during rhEPO treatment is low in children with renal anaemia; (2) rhEPO sensitivity of circulating BFU-E is not predictive for the in vivo response; (3) rhEPO treatment results in enhanced sensitivity of BFU-E to IL3.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02000786
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  • 3
    Electronic Resource
    Electronic Resource
    Circulating haematopoietic progenitors during treatment of renal anaemia with recombinant human erythropoietin (1994)
    Springer
    European journal of pediatrics 153 (1994), S. 43-48 
    Details
    ISSN: 1432-1076
    Keywords: Key words: Chronic renal failure – Erythropoietin – Haematopoietic progenitors – Haemodialysis – Interleukin 3
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. The effect of recombinant human erythropoietin (rhEPO) and interleukin 3 (IL3) on circulating haematopoietic progenitors consisting mainly of immature burst-forming-units-erythrocytes (BFU-E), was investigated in ten paediatric patients treated by regular haemodialysis. During a 30-week study rhEPO treatment resulted in a rise of median haemoglobin levels from 6.7 g/dl to 〉10 g/dl in all patients. Before initiating rhEPO treatment the number of circulating BFU-E in chronic renal failure patients responded to grading doses of rhEPO in vitro similar to that in control children; however, the dose-response curves were not predictive for the in vivo response to rhEPO. After an initial rise in five patients BFU-E numbers declined by week 30 of rhEPO treatment. BFU-E numbers decreased to 35% of pretreatment values. The number of granulocyte-macrophage colony forming cells (GM-CFC) also decreased during rhEPO treatment. Addition of IL3 to the culture medium containing saturating concentrations of granulocyte-macrophage colony stimulating factor did not stimulate BFU-E numbers of patients before rhEPO treatment or those of controls. However, 2 weeks after start of rhEPO treatment IL3 increased the growth of patient's BFU-E in vitro to 220% of pretreatment levels, followed by a gradual decrease of stimulation until the end of observation. These findings indicate that: (1) long-term recruitment of circulating haematopoietic progenitors during rhEPO treatment is low in children with renal anaemia; (2) rhEPO sensitivity of circulating BFU-E is not predictive for the in vivo response; (3) rhEPO treatment results in enhanced sensitivity of BFU-E to IL3.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02000786
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Dihydrofolate reductase-activity in brain tissue (1987)
    Springer
    Annals of hematology 55 (1987), S. 483-488 
    Details
    ISSN: 1432-0584
    Keywords: Dihydrofolate reductase ; Cranial irradiation ; Brain tissue
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The mechanism responsible for the toxic late effects of cranial irradiation, followed by the administration of systemic methotrexate (MTX) on brain tissue, is still under discussion. We studied the influence of X-irradiation on dihydrofolate reductase (E.C. 1.5.1.3) activity (DHFR), the enzyme inhibited by MTX. New Zealand white rabbits, 6–9 weeks old, underwent 24 Gy fractionated or 20 Gy single-dose brain irradiation using a 60Co source. Before, immediately following, and 1, 2, 4, 12 weeks after irradiation, DHFR was measured in brain and liver tissue by a photometric assay. DHFR in brain tissue was 11.9±2.9 mU/g wet weight (ww) × h and in liver tissue 121.8±24.2 mU/g ww × h. Fractionated brain irradiation with 2 Gy per day produced no significant changes in brain DHFR. Single-dose cranial irradiation significantly decreased brain DFHR (7.3±0.6 mU/g ww × h). Suppression of the developmental increase of DHFR by X-irradiation in young rabbits could be excluded by determining the unchanged brain-to-liver ratios of DHFR in the animals with fractionated brain irradiation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00320216
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  • 5
    Electronic Resource
    Electronic Resource
    Absorption process for the removal of organic solvents from exhaust air (1989)
    Weinheim : Wiley-Blackwell
    Chemical Engineering & Technology - CET 12 (1989), S. 63-70 
    Details
    ISSN: 0930-7516
    Keywords: Chemistry ; Industrial Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Process Engineering, Biotechnology, Nutrition Technology
    Notes: It is shown in this report that, under certain conditions, cleaning of exhaust air by absorption presents an interesting alternative to the existing methods. In particular, this process can be used with advantage for small quantities of exhaust air, high and strongly fluctuating solvent concentrations and solvent mixtures such as those occurring in batch operation. A washing agent was employed which had not been previously used for this purpose. Its properties, which were investigated in the laboratory, were confirmed during a 15-months' trial period in a semi-industrial plant. The process and plant were optimized for practical use. Through its option of solvent recovery from exhaust air, this physical absorption process contributes to environmental control.
    Additional Material: 10 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/ceat.270120111
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  • 6
    Electronic Resource
    Electronic Resource
    Reactions of Electrochemically Generated Organometallics, 2. The Use of Zinc, Tin, Aluminium, Indium, and Iron as Sacrificial Anodes in the Electrochemically Assisted Reformatsky Reaction of Ethyl 2-Bromoalkanoates with Succinic AnhydrideDedicated to Prof. Dr. Ernst Schmitz on the occasion of his 65th birthday. (1993)
    Weinheim : Wiley-Blackwell
    Berichte der deutschen chemischen Gesellschaft 126 (1993), S. 1247-1249 
    Details
    ISSN: 0009-2940
    Keywords: Reformatsky reaction, electrochemically assisted ; Succinic anhydride ; Sacrificial anodes ; Indium ; Hexanedicarboxylic acid, 3-oxo-2-substituted, 1-ethyl esters ; Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Notes: Zinc, tin, aluminium, indium, and iron were applied as sacrificial anodes in the electrochemically assisted Reformatsky reaction of succinic anhydride (1) with ethyl 2-bromoalkanoates 2. In all cases the expected 1-ethyl 3-oxohexanedioates 3 were abtained in good to moderate yields. However, the amount of current consumed for the dissolution of one equivalent of anode metal strongly depends on the kind of metal. Whereas aluminium and iron require 1 Faraday per equivalent (Faraday val-1), tin demands only 0.9 Faraday val-1, and zinc and indium require an even smaller amount of current. It is assumed that in the case of the last three metals the electro-chemical Reformatsky reaction is overlapped by a current-independent normal Reformatsky reaction, in which the anode acts as activated metal.
    Additional Material: 1 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/cber.19931260526
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