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Tiglicaciduria in propionicacidaemia.

W L Nyhan, T Ando, K Rasmussen

    The Biochemical Journal
    |February 1, 1972
    PubMed
    Summary
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    Tiglic acid was found in human urine for the first time in patients with propionicacidaemia, a condition linked to propionate oxidation defects. This finding suggests a potential metabolic pathway disruption in affected individuals.

    Area of Science:

    • Biochemistry
    • Metabolic disorders
    • Human physiology

    Background:

    • Propionicacidaemia is a rare genetic disorder affecting propionate metabolism.
    • A defect in propionate oxidation leads to the accumulation of various organic acids.
    • Tiglic acid has not been previously identified in human body fluids.

    Purpose of the Study:

    • To report the novel detection of tiglic acid in human urine.
    • To investigate the potential metabolic origin of tiglic acid in propionicacidaemia.
    • To explore the biochemical mechanisms underlying tiglic acid accumulation.

    Main Methods:

    • Urine analysis of patients with propionicacidaemia.
    • Biochemical pathway analysis.
    • Enzyme kinetics investigation.

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    Main Results:

    • Tiglic acid was detected in the urine of two patients with propionicacidaemia.
    • These patients exhibited a known defect in propionate oxidation.
    • A proposed mechanism involves competition between acrylyl-CoA and tiglyl-CoA for the enzyme crotonase.

    Conclusions:

    • The presence of tiglic acid in human urine is reported for the first time.
    • Tiglic acid accumulation in propionicacidaemia may result from a specific metabolic block.
    • Further research is needed to fully elucidate the role of tiglic acid in human metabolism.