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Related Experiment Videos

Demyelination in a transgenic mouse: a model for multiple sclerosis

F G Mastronardi1, C A Ackerley, L Arsenault

  • 1Department of Biochemistry, Hospital for Sick Children, Toronto, Canada.

Journal of Neuroscience Research
|October 15, 1993
PubMed
Summary

Transgenic mice with excess DM20 (a myelin protein) developed demyelination and neurological symptoms. This suggests persistent immature myelin in adults causes myelin disruption and disease.

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Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Myelin proteolipid protein (PLP) is crucial for myelin sheath integrity.
  • DM20 is a splice variant of PLP, abundant in young myelin.
  • Overexpression of myelin components can lead to neurological dysfunction.

Purpose of the Study:

  • To investigate the effects of high-level DM20 expression on myelin development and neurological function.
  • To determine if DM20 overexpression causes demyelination in a transgenic mouse model.

Main Methods:

  • Generated transgenic mice (ND4) with 70 copies of the DM20 transgene.
  • Clinical assessment of neurological symptoms (tremors, gait) at different ages.
  • Quantification of myelin content and PLP levels.

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  • Histological and electron microscopic analysis for demyelination, astrogliosis, and cellular infiltration.
  • Optic nerve examination.

    • Main Results:

    • ND4 mice showed normal development until 3 months, followed by tremors and death by 8-10 months.
    • Significant reduction in myelin content (17% of normal) by 10 months.
    • Progressive astrogliosis and evidence of disrupted myelin with astrocyte phagocytosis.
    • Lymphocytic infiltration observed.
    • Optic nerves showed denuded and thinly myelinated axons.

    Conclusions:

    • High DM20 expression leads to progressive demyelination and neurological deficits in adult mice.
    • Persistence of immature myelin composition into adulthood results in myelin instability.
    • Demyelination in ND4 mice is linked to the failure of myelin maturation.