The myelin mutant taiep rat as a model for developmental brain disorders

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Abstracto

  • Central nervous system (CNS) pathologies produced substantial health burdens because is the first cause of disability-adjusted life years, but less is known about the burden of myelin diseases, among them leukodystrophies. The myelin-mutant taiep rat has a mutation in the TUBB4A gene, which encodes β-tubulin 4A, primarily expressed in oligodendrocytes, and had an initial hypomyelination followed by a progressive demyelination of the CNS. The oligodendrocytes in taiep rats present an accumulation of microtubules in their cytoplasm and its myelin processes that disrupt transporting mechanisms, reducing protein and lipid contents on myelin sheets. We showed that taiep rats have similar weight, size, incisive emergence, and opening of the eyes, as well as the righting reflex, cliff avoidance, and negative geotaxis with respect to Sprague Dawley rats. However, the speed of gait is significantly reduced in taiep from 21 to 28 days of age, supporting early alterations in locomotor mechanisms for this myelin-mutant with tubulinopathy.

fecha de publicación

  • enero 2021

Palabras clave

  • Adrenoleukodystrophy
  • Animal models
  • Axon
  • Demyelination
  • Hypomyelination
  • Leukodystrophy
  • Locomotion
  • Multiple sclerosis
  • Tubulinopathy

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