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Metabolic Brain Disease

, Volume 34, Issue 1, pp 367–372 | Cite as

FOXRED1 silencing in mice: a possible animal model for Leigh syndrome

  • Mohamed SalamaEmail author
  • Sara El-Desouky
  • Aziza Alsayed
  • Mahmoud El-Hussiny
  • Abdelrahman Moustafa
  • Yasmeen Taalab
  • Wael Mohamed
Short Communication
  • 209 Downloads

Abstract

Leigh syndrome (LS) is one of the most puzzling mitochondrial disorders, which is also known as subacute necrotizing encephalopathy. It has an incidence of 1 in 77,000 live births worldwide with poor prognosis. Currently, there is a poor understanding of the underlying pathophysiological mechanisms of the disease without any available effective treatment. Hence, the inevitability for developing suitable animal and cellular models needed for the development of successful new therapeutic modalities. In this short report, we blocked FOXRED1 gene with small interfering RNA (siRNA) using C57bl/6 mice. Results showed neurobehavioral changes in the injected mice along with parallel degeneration in corpus striatum and sparing of the substantia nigra similar to what happen in Leigh syndrome cases. FOXRED1 blockage could serve as a new animal model for Leigh syndrome due to defective CI, which echoes damage to corpus striatum and affection of the central dopaminergic system in this disease. Further preclinical studies are required to validate this model.

Keywords

FOXRED1 Neurodegenerative diseases Leigh syndrome Gene silencing 

Notes

Acknowledgments

This work was supported by a grant from the Egyptian Science and Technology Development Fund (STDF) through Basic and Applied Research Grants (BARG) program, grant number (13892) [MS].

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Mohamed Salama
    • 1
    • 2
    Email author
  • Sara El-Desouky
    • 1
  • Aziza Alsayed
    • 1
  • Mahmoud El-Hussiny
    • 1
  • Abdelrahman Moustafa
    • 1
  • Yasmeen Taalab
    • 2
  • Wael Mohamed
    • 3
    • 4
  1. 1.Medical Experimental Research Center (MERC), Faculty of MedicineMansoura UniversityMansouraEgypt
  2. 2.Toxicology Department, Faculty of MedicineMansoura UniversityMansouraEgypt
  3. 3.Clinical Pharmacology Department, Faculty of MedicineMenoufia UniversityShebeen El-KomEgypt
  4. 4.Department of Basic Medical Science, Kulliyyah of MedicineInternational Islamic UniversityKuantanMalaysia

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