Introduction:
Demyelinating diseases of the central nervous system (CNS) comprise a
group of neurological disorders characterized by progressive (and eventually irreversible) loss of
oligodendrocytes and myelin sheaths in the white matter tracts. Some of myelin disorders include:
Multiple sclerosis, Guillain-Barré syndrome, peripheral nerve polyneuropathy and others. To date,
the etiology of these disorders is not well known and no effective treatments are currently available
against them. Therefore, further research is needed to gain a better understand and treat these
patients. To accomplish this goal, it is necessary to have appropriate animal models that closely
resemble the pathophysiology and clinical signs of these diseases. Herein, we describe the model of
toxic demyelination induced by cuprizone (CPZ), a copper chelator that reduces the cytochrome
and monoamine oxidase activity into the brain, produces mitochondrial stress and triggers the local
immune response. These biochemical and cellular responses ultimately result in selective loss of
oligodendrocytes and microglia accumulation, which conveys to extensive areas of demyelination
and gliosis in corpus callosum, superior cerebellar peduncles and cerebral cortex. Remarkably,
some aspects of the histological pattern induced by CPZ are similar to those found in multiple
sclerosis. CPZ exposure provokes behavioral changes, impairs motor skills and affects mood as that
observed in several demyelinating diseases. Upon CPZ removal, the pathological and histological
changes gradually revert. Therefore, some authors have postulated that the CPZ model allows to
partially mimic the disease relapses observed in some demyelinating diseases.
Conclusion:
for five decades, the model of CPZ-induced demyelination is a good experimental
approach to study demyelinating diseases that has maintained its validity, and is a suitable pharmacological
model for reproducing some key features of demyelinating diseases, including multiple sclerosis.
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