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Histochemische und biochemische Untersuchungen bei Morbus Gaucher

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Zusammenfassung

1. Bei Morbus Gaucher konnte durch histochemische Testung der Gaucher-Zellen und mittels Knochenmarksbiopsie eine Verbesserung der Diagnostik erreicht werden. Besonders charakteristisch ist der Gehalt an PAS-positiven Substanzen und sauren Phosphatasen.

2. Die Aktivität der sauren Phosphatasen in Serum und Plasma steigt bis durchschnittlich 20 KAE an. Das Enzym läßt sich durch Stärkegelelektrophorese in fünf Fraktionen auftrennen, das Enzymgemisch hat pH-Optima bei pH 3,85 und 5,1, ist wenig substratspezifisch und verhält sich gegen Aktivatoren und Inhibitoren anders als Erythrocyten-, Thrombocytenund Prostataphosphatasen.

3. Die Aktivität der sauren Phosphatasen im Milzhomogenat ist bei M. Gaucher etwa zehmal höher als in Normalmilz; die Milzphosphatasen haben dieselben Eigenschaften wie die Plasmaphosphatasen. Dies deutet auf eine enge Beziehung zwischen den in den Geweben gespeicherten und den Plasmaphosphatasen hin.

Summary

1. Histochemical investigations of storage cells and bone marrow biopsy proved to be a valuable diagnostic procedure in patients with Gaucher's disease. Special characteristics were strong positive PAS and acid phosphatase reaction of the plasma of Gaucher cells.

2. The activity of acid phosphatase of serum and “thrombocyte free” plasma was increased approximately to 20 King-Armstrong units. The enzyme was separated by starch gel electrophoresis into 5 fractions. The enzyme mixture had optimal activity at pH 3,85 and 5,1, showed minimal substrate specificity and reacted differently from erythrocyte thrombocyte and prostate acid phosphatases under influence of various activating and inhibiting chemicals.

3. The acid phosphatase activity in a homogenate of the spleen of a patient suffering from Gaucher's disease was increased 10 times against a control spleen. Properties of spleen acid phosphatases were similar as compared with acid phosphatases of the plasma. These facts point toward a strong correlation of tissue and plasma acid phosphatases in Gaucher's disease.

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Authors and Affiliations

  1. Aus der I. Medizinischen Klinik der Universität Wien, Austria

    H. Czitober, E. Gründig & B. Schobel

  2. dem Med.-Chem. Institut der Universität Wien, Austria

    H. Czitober, E. Gründig & B. Schobel

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  1. H. Czitober
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  2. E. Gründig
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  3. B. Schobel
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Czitober, H., Gründig, E. & Schobel, B. Histochemische und biochemische Untersuchungen bei Morbus Gaucher. Klin Wochenschr 42, 1179–1184 (1964). https://doi.org/10.1007/BF01533622

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