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  • 1
    Electronic Resource
    Electronic Resource
    Über eine neue erythrocytäre Glucose-6-Phosphatdehydrogenase-Variante, ‚Typ Frankfurt‘ (1974)
    Springer
    Journal of molecular medicine 52 (1974), S. 478-484 
    Details
    ISSN: 1432-1440
    Keywords: New erythrocyte G-6-PD variant ; kinetical parameters ; Neue erythrocytäre G-6-PD-Variante ; Enzymkinetische Parameter
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über Untersuchungen zur Charakterisierung einer neuen erythrocytären G-6-PD-Variante, die in einer deutschen Sippe gefunden wurde, berichtet. Nachdem bereits vorausgegangene Familienuntersuchungen den allosomalen an das X-Chromosom gebundenen Erbgang aufgezeigt hatten, wurde jetzt aus den Erythrocyten eines hemizygoten Erbmalsträgers dieser Sippe das Enzym teilweise gereinigt und angereichert; in dieser Enzymanreicherung wurden die Aktivität, die elektrophoretische Wanderungsgeschwindigkeit, die Michaeliskonstante für G-6-P und NADP, die relative Utilisationsrate von 2-d-G-6-P und Gal-6-P, die Thermostabilität, die Aktivierung durch Hitze und die pH-Optima untersucht. Der Vergleich dieser Untersuchungsergebnisse mit den aus der Literatur bekannten Werten von 117 weiteren G-6-PD-Varianten und mit den eigenen Ergebnissen von parallel-laufenden Untersuchungen am normalen Enzymtyp Gd B zeigte, daß es sich bei dem untersuchten Enzym um eine neue Variante handelt, die den Empfehlungen der WHO entsprechend als Typ Frankfurt bezeichnet worden ist.
    Notes: Summary The paper reports on the results of investigations on the characterization of a new erythrocyte G-6-PD variant, which was found in a German family. Earlier family investigations had already revealed an allosomal X-chromosome linked heredity. The enzyme was isolated and purified from erythrocytes of a hemizygous carrier of the enzyme defect. This enzyme preparation was used for the determination of the following parameters: enzyme activity, electrophoretic mobility, Michaelis constants for glucose-6-phosphate and NADP, relative rate of utilisation for 2-deoxyglucose-6-phosphate and galactose-6-phosphate, thermostability, thermo-activation, and pH-optima. The results were compared with the known data of 117 other G-6-PD variants and with own results of parallel studies on the normal enzyme Gd B. The enzyme in question could be shown to represent a new variant. According to the WHO recommendations this variant was named type Frankfurt (Gd Frankfurt).
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01468536
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  • 2
    Electronic Resource
    Electronic Resource
    Über eine neue erythrocytäre Glucose-6-Phosphatdehydrogenase-Variante, ‚Typ Frankfurt‘ (1974)
    Springer
    Journal of molecular medicine 52 (1974), S. 485-492 
    Details
    ISSN: 1432-1440
    Keywords: New erythrocyte G-6-PD variant ; Gd Frankfurt ; Structural abnormality ; Molecular weight ; Peptide analysis ; Amino acid substitution ; Lys → Glu ; Neue erythrocytäre G-6-PD-Variante ; Gd Frankfurt ; strukturelle Anomalie ; Molekulargewicht ; Peptidanalyse ; Aminosäurenaustausch ; Lys → Glu
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Eine neue, anomale erythrocytäre G-6-PD-Variante, Gd Frankfurt, wurde nach chromatographischer Reingung auf DEAE-Cellulose, durch fraktionierte Ammoniumsulfat-Fällung und zweimalige Gelfiltration auf das rund 14000fache angereichert. Mittels Gelfiltration wurde eine Molekulargewichtsbestimmung durchgeführt, ferner wurde der SH-Gruppen-Gehalt und die Hemmung der Aktivität durch N-Äthylmaleimid bestimmt. Im Vergleich zum Normalenzym, Gd B, fand sich — bei gleichem SH-Gruppen-Gehalt — ein von der Norm nur geringfügig abweichendes Molekulargewicht von 223 000 gegenüber 243 500 beim Normalenzym und eine etwas stärkere Hemmung durch N-Äthylmaleimid. Nach tryptischer Verdauung ergab die Peptidanalyse mittels Fingerprint-Verfahrens auf Kieselgel-S-Dünnschichtplatten ein abweichendes Verhalten für 1 Peptid, während die restlichen 39 Peptide die gleiche Lokalisation aufwiesen. Die Aminosäuren-Analyse dieser differienden Peptide deckte für die neue Variante einen Austausch von Lysin gegen Glutaminsäure auf.
    Notes: Summary A new, abnormal erythrocyte G-6-PD variant, Gd Frankfurt, was first purified chromatographically on DEAE cellulose. Further purification was performed by fractionate precipitation with ammonium sulfate and by double gel filtration on Sephadex G-200. By these procedures a nearly 14000-fold concentration was obtained. In this preparation as well as in a control preparation of the normal enzyme variant Gd B the molecular weight, the content of sulfhydryl groups and the inhibition of enzyme activity by N-ethylmaleimide was determined. The content of sulfhydryl groups was found to be the same as in the normal enzyme variant. The molecular weights are slightly different: for the abnormal enzyme variant the value obtained was 223 000, and 243 500 for the normal enzyme. The inhibition by N-ethyl-maleimide showed to be somewhat stronger in the abnormal variant than in the normal enzyme. Peptide analysis after tryptic digestion was performed by fingerprint technique on silica gel-starch thin layers. 39 peptides of the abnormal variant showed the same pattern as in the normal enzyme whereas one peptide was found in a differing localization. Amino acid analysis' of the differing peptides revealed a substitution of glutamic acid for lysine in the abnormal enzyme variant.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01468537
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Principles and present status of a prospective multicenter study on the clinical relevance of the kiel classification (1981)
    Springer
    Annals of hematology 43 (1981), S. 155-166 
    Details
    ISSN: 1432-0584
    Keywords: Non-Hodgkin lymphoma ; Kiel classification ; Low-grade malignancy ; High-grade malignancy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary On the basis of the retrospective analysis of 405 patients, suggesting the clinical relevance of the Kiel classification of non-Hodgkin lymphomas (NHL), a prospective multicenter study was started on October 1st, 1975, by the Kiel Lymphoma Study Group in order to further clarify the clinical and prognostic features of the different lymphoma entities defined by this histopathologic scheme. Diagnostic protocol provides initial staging evaluation according to a modification of the Ann Arbor classification. Therapeutic approach is based on the hypothesis that, like Hodgkin's disease, NHL originate, at least in part, as localized lymphatic or extralymphatic tumors. Thus, extended field irradiation is performed in stages I and II (except for lymphoblastic lymphoma in children and young adults) whereas in the more advanced stages III and IV (except for stage III of centroblastic-centrocytic lymphoma) chemotherapy with additional radiotherapy is applied. Until June 1979, 815 patients entered the study (69.7% with NHL of low-grade malignancy). For the interim evaluation underlying the present and the other papers of this series data of 511 patients were available. Survival of patients with NHL of low-grade malignancy significantly exceeds that of patients with NHL of high-grade malignancy. NHL with good prognosis such as chronic lymphocytic leukaemia and centroblastic-centrocytic lymphoma can be differentiated from NHL with a poor course such as lymphoblastic and immunoblastic lymphomas. In addition, the existence of a third group with an intermediate prognosis comprising centrocytic and centroblastic lymphomas and, possibly, also LP immunocytoma is suggested. However, different initial slope of survival curves shows that this latter group of NHL is not homogeneous with regard to prognosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00363886
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  • 4
    Electronic Resource
    Electronic Resource
    Clinical and prognostic heterogeneity of non-hodgkin lymphomas of high-grade malignancy (1981)
    Springer
    Annals of hematology 43 (1981), S. 201-211 
    Details
    ISSN: 1432-0584
    Keywords: Non-Hodgkin lymphoma ; High-grade malignancy ; Lymphoblastic lymphoma ; Immunoblastic lymphoma ; Centroblastic lymphoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Comparison of clinical data of 64 patients with centroblastic lymphoma, 55 patients with immunoblastic lymphoma and 31 patients with lymphoblastic lymphoma not only confirmed the original assumption of high-grade malignancy as proposed by the concept of the Kiel classification but also demonstrated distinct clinical differences, particularly between lymphoblastic lymphoma and the two other entities. Rapid lymph node enlargement as well as steep fall of survival curves within the first year after diagnosis were common characteristics. Bimodal age distribution, predominance of males and early generalization of disease were typical features of lymphoblastic lymphoma; elderly patients and patients with the unclassified subtypes of lymphoblastic lymphoma exhibited the worst prognosis. Whereas patients with centroblastic and immunoblastic lymphomas showed similar distribution of age, sex and initial stage of disease, patients with immunoblastic lymphoma presented more frequently with a reduced performance status and showed a poorer response to radio- and chemotherapy resulting in a worse prognosis discernible after the first year of follow-up. Generalization during course of the disease was significantly more frequent in immunoblastic than in centroblastic lymphoma.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00363890
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    Paper (German National Licenses)
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