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A Non-random Mouse Model for Pharmacological Reactivation of Mecp2 on the Inactive X Chromosome
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Genética
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JoVE Journal Genética
A Non-random Mouse Model for Pharmacological Reactivation of Mecp2 on the Inactive X Chromosome

A Non-random Mouse Model for Pharmacological Reactivation of Mecp2 on the Inactive X Chromosome

DOI:
10.3791/59449-v

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08:27 min

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May 22, 2019

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, , ,
1Department of Biochemistry and Molecular Genetics,University of Virginia School of Medicine, 2Department of Neuroscience,University of Virginia School of Medicine

Capítulos

  • 00:04Título
  • 00:36Mouse Preparation
  • 01:12Pharmacological Inactive X Chromosome (Xi) Reactivation
  • 02:50Brain Harvest
  • 04:45Cryo-sectioning
  • 05:35Xi Reactivation Determination
  • 06:34Results: Representative Xi-Mecp2 Validation and Pharmacological Reactivation
  • 07:52Conclusion

Summary

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Here, we describe a protocol to generate a viable female murine model with non-random X chromosome inactivation, i.e., the maternally-inherited X chromosome is inactive in 100% of the cells. We also describe a protocol to test feasibility, tolerability, and safety of pharmacological reactivation of the inactive X chromosome in vivo.

Tags

Non-random Mouse ModelMecp2Inactive X ChromosomeXi ReactivationStereotactic InjectionChemical InhibitorBrainNeurociênciaX-linked Disease ModelsDDX3X Syndrome

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