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46714

A Novel Animal Model Prepared by Blocking (FOXRED1 gene) for neuro-toxicological studies of Leigh Syndrome

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Last updated: 30 Jan 2023

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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 substantianigra similar to what happen in Leigh syndrome cases. FOXRED1 blockage could serve as a new animal model for neuro-toxicological studies of Leigh syndrome due to defective mitochondrial complex I (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.

DOI

10.21608/mjfmct.2019.46714

Keywords

FOXRED1, Neurodegenerative diseases, Leigh syndrome, gene silencing

Authors

First Name

Mohamed

Last Name

Salama

MiddleName

-

Affiliation

Forensic Medicine and Clinical Toxicology Department, Faculty of Medicine, Mansoura University, Egypt/ Medical Experimental Research Center (MERC), Faculty of Medicine, Mansoura University, Egypt.

Email

toxicsalama@hotmail.com

City

-

Orcid

-

First Name

Sara

Last Name

El-Desouky

MiddleName

-

Affiliation

Medical Experimental Research Center (MERC), Faculty of Medicine, Mansoura University, Egypt.

Email

-

City

-

Orcid

-

First Name

Aziza

Last Name

Alsayed

MiddleName

-

Affiliation

Medical Experimental Research Center (MERC), Faculty of Medicine, Mansoura University, Egypt.

Email

-

City

-

Orcid

-

First Name

Mahmoud

Last Name

El-Hussiny

MiddleName

-

Affiliation

Medical Experimental Research Center (MERC), Faculty of Medicine, Mansoura University, Egypt.

Email

-

City

-

Orcid

-

First Name

Abdelrahman

Last Name

Moustafa

MiddleName

-

Affiliation

Medical Experimental Research Center (MERC), Faculty of Medicine, Mansoura University.

Email

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City

-

Orcid

-

First Name

Emad

Last Name

Fekry

MiddleName

-

Affiliation

Forensic Medicine and Clinical Toxicology Department, Faculty of Medicine, Mansoura University, Egypt

Email

dremadfekry@yahoo.com

City

-

Orcid

-

First Name

Yasmeen

Last Name

Taalab

MiddleName

-

Affiliation

Forensic Medicine and Clinical Toxicology Department, Faculty of Medicine, Mansoura University. (

Email

-

City

-

Orcid

-

First Name

Wael

Last Name

Mohamed

MiddleName

-

Affiliation

Clinical Pharmacology Department, Faculty of Medicine, Menoufia University; Egypt

Email

-

City

-

Orcid

-

Volume

27

Article Issue

1

Related Issue

7043

Issue Date

2019-01-01

Receive Date

2019-09-01

Publish Date

2019-01-01

Page Start

77

Page End

86

Print ISSN

1110-5437

Online ISSN

2682-3217

Link

https://mjfmct.journals.ekb.eg/article_46714.html

Detail API

https://mjfmct.journals.ekb.eg/service?article_code=46714

Order

6

Type

Original Article

Type Code

966

Publication Type

Journal

Publication Title

Mansoura Journal of Forensic Medicine and Clinical Toxicology

Publication Link

https://mjfmct.journals.ekb.eg/

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Details

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Article

Created At

22 Jan 2023