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Cycloleucine encephalopathy.

C M Greco, H C Powell, R S Garrett

    Neuropathology and Applied Neurobiology
    |September 1, 1980
    PubMed
    Summary
    This summary is machine-generated.

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    Cycloleucine causes spongiform changes in the brain

    Area of Science:

    • Neuroscience
    • Biochemistry
    • Toxicology

    Background:

    • Cycloleucine is a non-metabolizable amino acid analogue.
    • Amino acid transport and metabolism are crucial for brain function.

    Purpose of the Study:

    • To investigate the mechanisms by which cycloleucine induces spongiform changes in the brain.
    • To characterize the resulting astrocyte lesions and myelinopathy.

    Main Methods:

    • Administration of cycloleucine to rats and mice.
    • Electron microscopy of cerebral white matter.
    • Analysis of biochemical pathways affected by cycloleucine.

    Main Results:

    • Cycloleucine induced status spongiosus and astrocyte lesions in the cerebral white matter.

    Related Experiment Videos

  • Mechanisms include competition for amino acid transport, rRNA maturation interference, and blocked transmethylation.
  • Spongiform changes in myelin sheaths were observed, characterized by separated lamellae and astrocyte filament whorls.
  • Kidney effects included aminoaciduria.
  • Myelinopathy was dose-dependent and cumulative due to cycloleucine's long half-life.

    • Conclusions:

    • Cycloleucine causes a dose-dependent spongiform myelinopathy through multiple biochemical disruptions.
    • Findings provide insights into other aminoaciduria-related spongiform myelinopathies.