FOXRED1 silencing in mice: a possible animal model for Leigh syndrome.
FOXRED1
Gene silencing
Leigh syndrome
Neurodegenerative diseases
Journal
Metabolic brain disease
ISSN: 1573-7365
Titre abrégé: Metab Brain Dis
Pays: United States
ID NLM: 8610370
Informations de publication
Date de publication:
02 2019
02 2019
Historique:
received:
08
06
2018
accepted:
30
10
2018
pubmed:
6
11
2018
medline:
7
6
2019
entrez:
5
11
2018
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 30392038
doi: 10.1007/s11011-018-0334-z
pii: 10.1007/s11011-018-0334-z
doi:
Substances chimiques
Molecular Chaperones
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
367-372Commentaires et corrections
Type : CommentIn
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