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FOXRED1 silencing in mice: a possible animal model for leigh syndrome.

Mohamed, Salama and El-Desouky, Sara and Alsayed, Aziza and El-Hussiny, Mahmoud and Moustafa, Abdelrahman and Taalab, Yasmeen M. and Mohamed, Wael Mohamed Yousef (2019) FOXRED1 silencing in mice: a possible animal model for leigh syndrome. Metabolic Brain Disease, 34 (1). pp. 367-372. ISSN 08857490

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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.

Item Type: Article (Journal)
Additional Information: 7497/67455
Uncontrolled Keywords: FOXRED1 . Neurodegenerative diseases . Leigh syndrome . Gene silencin
Subjects: R Medicine > R Medicine (General)
Kulliyyahs/Centres/Divisions/Institutes: Kulliyyah of Medicine
Kulliyyah of Medicine > Department of Basic Medical
Depositing User: Dr Wael Mohamed
Date Deposited: 15 Nov 2018 17:14
Last Modified: 03 May 2019 10:54
URI: http://irep.iium.edu.my/id/eprint/67455

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