Klinische Wochenschrift

, Volume 43, Issue 2, pp 93–97 | Cite as

Die Ausscheidung von β-Aminoisobuttersäure bei Gesunden und Kranken

  • H. Brunschede
  • R. Hoffbauer
  • H. W. Goedde
Originalien

Zusammenfassung

1. Bei größerer interindividueller Schwankung zeigt die Ausscheidung von β-AIB im Urin eine erstaunliche intraindividuelle Konstanz.

2. Untersuchungen von Leberkranken ergaben, daß der Funktionszustand der Leber keinen wesentlichen Einfluß auf die Ausscheidung von β-AIB im Urin hat.

3. Die Hälfte der untersuchten Patienten mit Hämochromatose scheidet größere Mengen dieser Aminosäure aus.

4. Der bei der Ahorn-Sirup-Krankheit auftretende Enzymdefekt (Ausfall der α-Ketosäureoxydasen) hat keinen Einfluß auf die Menge der eliminierten β-AIB.

Abkürzungen

β-AIB

β-Aminoisobuttersäure

DNFB

2,4 Dinitro-1-Fluorobenzol

Summary

1. β-AIB excretion varies greatly from individual to individual, whereas normal day to day differences in β-AIB excretion rates for any one person appear to remain constant.

2. Impaired liver function does not markedly alter urinary β-AIB excretion.

3. Half of our patients with hemochromatosis excrete large amounts of β-AIB.

4. Impaired activities of α-ketoacid-oxydases in maple syrup urine disease have no apparent effect on β-AIB excretion.

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Copyright information

© Springer-Verlag 1965

Authors and Affiliations

  • H. Brunschede
    • 1
  • R. Hoffbauer
    • 1
  • H. W. Goedde
    • 1
  1. 1.Aus der Abteilung für Biochemische Genetik, Anthropologisches Institut der Universität Freiburg i. Br.Germany

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