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Related Experiment Videos

Golgi study on macular mutant mouse after copper therapy.

H Kawasaki1, T Yamano, S Iwane

  • 1Department of Pediatrics, Shiga University of Medical Science, Japan.

Acta Neuropathologica
|January 1, 1988
PubMed
Summary

Copper therapy improved Menkes kinky hair disease (MKHD) mouse models by correcting clinical symptoms and enhancing neuropathological changes, particularly in the cerebellum. This treatment offers potential for managing this genetic disorder.

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Area of Science:

  • Neuroscience
  • Genetics
  • Biochemistry

Background:

  • Menkes kinky hair disease (MKHD) is a genetic disorder affecting copper metabolism.
  • The macular mutant mouse hemizygote serves as a model for MKHD.
  • Neuropathological deficits are a hallmark of MKHD.

Purpose of the Study:

  • To investigate the effects of copper administration on a mouse model of MKHD.
  • To assess both clinical and neuropathological outcomes following copper treatment.
  • To determine the efficacy of copper therapy in ameliorating MKHD symptoms.

Main Methods:

  • Intraperitoneal injections of cupric chloride were administered to macular mutant mouse hemizygotes at specific dosages and intervals.
  • Clinical observations included monitoring whisker morphology, seizures, ataxia, and body weight.

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  • Neuropathological analysis focused on dendritic arborization of pyramidal neurons in the cerebral cortex and Purkinje cells in the cerebellum.

    • Main Results:

    • Copper treatment led to the straightening of curly whiskers, disappearance of seizures and ataxia, and increased body weight in the treated hemizygotes.
    • While dendritic arborization improved over time in both normal and treated mice, it remained poorer in treated hemizygotes compared to controls.
    • Abnormal Purkinje cells in the cerebellum showed improvement, though focal dendritic swellings persisted.

    Conclusions:

    • Copper therapy effectively ameliorates clinical manifestations in the MKHD mouse model.
    • Neuropathological changes, especially in the cerebellum, are partially reversed by copper administration.
    • Copper treatment demonstrates therapeutic potential for Menkes kinky hair disease.