FOXRED1 silencing in mice: a possible animal model for Leigh syndrome
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...
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| Veröffentlicht in: | Metabolic brain disease 2019-02, Vol.34 (1), p.367-372 |
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| creator | Salama, Mohamed El-Desouky, Sara Alsayed, Aziza El-Hussiny, Mahmoud Moustafa, Abdelrahman Taalab, Yasmeen Mohamed, Wael |
| description | 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. |
| doi_str_mv | 10.1007/s11011-018-0334-z |
| format | Article |
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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.</description><identifier>ISSN: 0885-7490</identifier><identifier>EISSN: 1573-7365</identifier><identifier>DOI: 10.1007/s11011-018-0334-z</identifier><identifier>PMID: 30392038</identifier><language>eng</language><publisher>New York: Springer US</publisher><subject>Animal models ; Animals ; Biochemistry ; Biomedical and Life Sciences ; Biomedicine ; Biosynthesis ; Brain research ; Corpus striatum ; Corpus Striatum - pathology ; Degeneration ; Disease Models, Animal ; Dopamine receptors ; Echoes ; Encephalopathy ; Gene Silencing ; Genes ; Laboratory animals ; Leigh Disease - genetics ; Leigh Disease - pathology ; Medical treatment ; Medicine ; Metabolic Diseases ; Mice ; Mitochondria ; Molecular Chaperones - genetics ; Mutation ; Neostriatum ; Neurology ; Neurosciences ; Oncology ; Parkinson's disease ; Proteins ; Ribonucleic acid ; RNA ; Short Communication ; siRNA ; Substantia nigra ; Substantia Nigra - pathology</subject><ispartof>Metabolic brain disease, 2019-02, Vol.34 (1), p.367-372</ispartof><rights>Springer Science+Business Media, LLC, part of Springer Nature 2018</rights><rights>Metabolic Brain Disease is a copyright of Springer, (2018). All Rights Reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c372t-3d36f9a81306f118f1b31c53c3846d206648b011fce4606d202dd65629225ba53</citedby><cites>FETCH-LOGICAL-c372t-3d36f9a81306f118f1b31c53c3846d206648b011fce4606d202dd65629225ba53</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s11011-018-0334-z$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s11011-018-0334-z$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,776,780,27901,27902,41464,42533,51294</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/30392038$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Salama, Mohamed</creatorcontrib><creatorcontrib>El-Desouky, Sara</creatorcontrib><creatorcontrib>Alsayed, Aziza</creatorcontrib><creatorcontrib>El-Hussiny, Mahmoud</creatorcontrib><creatorcontrib>Moustafa, Abdelrahman</creatorcontrib><creatorcontrib>Taalab, Yasmeen</creatorcontrib><creatorcontrib>Mohamed, Wael</creatorcontrib><title>FOXRED1 silencing in mice: a possible animal model for Leigh syndrome</title><title>Metabolic brain disease</title><addtitle>Metab Brain Dis</addtitle><addtitle>Metab Brain Dis</addtitle><description>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.</description><subject>Animal models</subject><subject>Animals</subject><subject>Biochemistry</subject><subject>Biomedical and Life Sciences</subject><subject>Biomedicine</subject><subject>Biosynthesis</subject><subject>Brain research</subject><subject>Corpus striatum</subject><subject>Corpus Striatum - pathology</subject><subject>Degeneration</subject><subject>Disease Models, Animal</subject><subject>Dopamine receptors</subject><subject>Echoes</subject><subject>Encephalopathy</subject><subject>Gene Silencing</subject><subject>Genes</subject><subject>Laboratory animals</subject><subject>Leigh Disease - genetics</subject><subject>Leigh Disease - pathology</subject><subject>Medical treatment</subject><subject>Medicine</subject><subject>Metabolic Diseases</subject><subject>Mice</subject><subject>Mitochondria</subject><subject>Molecular Chaperones - genetics</subject><subject>Mutation</subject><subject>Neostriatum</subject><subject>Neurology</subject><subject>Neurosciences</subject><subject>Oncology</subject><subject>Parkinson's disease</subject><subject>Proteins</subject><subject>Ribonucleic acid</subject><subject>RNA</subject><subject>Short Communication</subject><subject>siRNA</subject><subject>Substantia nigra</subject><subject>Substantia Nigra - pathology</subject><issn>0885-7490</issn><issn>1573-7365</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNp1kE1LAzEQhoMotn78AC8S8Lw6k9lks96ktioUCqLgLexHtm7Zj5q0h_bXm9KqJ08DM--8887D2BXCLQIkdx4RECNAHQFRHG2P2BBlQlFCSh6zIWgtoyROYcDOvF8AAElMT9mAgFIBpIdsPJl9vI4fkfu6sV1Rd3Ned7ytC3vPM77sva_zxvKsq9us4W1f2oZXveNTW88_ud90petbe8FOqqzx9vJQz9n7ZPw2eo6ms6eX0cM0KigRq4hKUlWaaSRQFaKuMCcsJBWkY1UKUCrWefioKmysYNcRZamkEqkQMs8knbObve_S9V9r61dm0a9dF04agUKnQkulgwr3qsKF_M5WZulCfLcxCGYHzuzBmQDO7MCZbdi5Pjiv89aWvxs_pIJA7AU-jLq5dX-n_3f9BlQOddA</recordid><startdate>20190201</startdate><enddate>20190201</enddate><creator>Salama, Mohamed</creator><creator>El-Desouky, Sara</creator><creator>Alsayed, Aziza</creator><creator>El-Hussiny, Mahmoud</creator><creator>Moustafa, Abdelrahman</creator><creator>Taalab, Yasmeen</creator><creator>Mohamed, Wael</creator><general>Springer US</general><general>Springer Nature B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7TK</scope><scope>7U7</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>88G</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>C1K</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>M2M</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>PSYQQ</scope><scope>Q9U</scope></search><sort><creationdate>20190201</creationdate><title>FOXRED1 silencing in mice: a possible animal model for Leigh syndrome</title><author>Salama, Mohamed ; El-Desouky, Sara ; Alsayed, Aziza ; El-Hussiny, Mahmoud ; Moustafa, Abdelrahman ; Taalab, Yasmeen ; Mohamed, Wael</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c372t-3d36f9a81306f118f1b31c53c3846d206648b011fce4606d202dd65629225ba53</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>Animal models</topic><topic>Animals</topic><topic>Biochemistry</topic><topic>Biomedical and Life Sciences</topic><topic>Biomedicine</topic><topic>Biosynthesis</topic><topic>Brain research</topic><topic>Corpus striatum</topic><topic>Corpus Striatum - pathology</topic><topic>Degeneration</topic><topic>Disease Models, Animal</topic><topic>Dopamine receptors</topic><topic>Echoes</topic><topic>Encephalopathy</topic><topic>Gene Silencing</topic><topic>Genes</topic><topic>Laboratory animals</topic><topic>Leigh Disease - genetics</topic><topic>Leigh Disease - pathology</topic><topic>Medical treatment</topic><topic>Medicine</topic><topic>Metabolic Diseases</topic><topic>Mice</topic><topic>Mitochondria</topic><topic>Molecular Chaperones - genetics</topic><topic>Mutation</topic><topic>Neostriatum</topic><topic>Neurology</topic><topic>Neurosciences</topic><topic>Oncology</topic><topic>Parkinson's disease</topic><topic>Proteins</topic><topic>Ribonucleic acid</topic><topic>RNA</topic><topic>Short Communication</topic><topic>siRNA</topic><topic>Substantia nigra</topic><topic>Substantia Nigra - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Salama, Mohamed</creatorcontrib><creatorcontrib>El-Desouky, Sara</creatorcontrib><creatorcontrib>Alsayed, Aziza</creatorcontrib><creatorcontrib>El-Hussiny, Mahmoud</creatorcontrib><creatorcontrib>Moustafa, Abdelrahman</creatorcontrib><creatorcontrib>Taalab, Yasmeen</creatorcontrib><creatorcontrib>Mohamed, Wael</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Neurosciences Abstracts</collection><collection>Toxicology Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Psychology Database (Alumni)</collection><collection>ProQuest Pharma Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>ProQuest Psychology</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>ProQuest One Psychology</collection><collection>ProQuest Central Basic</collection><jtitle>Metabolic brain disease</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Salama, Mohamed</au><au>El-Desouky, Sara</au><au>Alsayed, Aziza</au><au>El-Hussiny, Mahmoud</au><au>Moustafa, Abdelrahman</au><au>Taalab, Yasmeen</au><au>Mohamed, Wael</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>FOXRED1 silencing in mice: a possible animal model for Leigh syndrome</atitle><jtitle>Metabolic brain disease</jtitle><stitle>Metab Brain Dis</stitle><addtitle>Metab Brain Dis</addtitle><date>2019-02-01</date><risdate>2019</risdate><volume>34</volume><issue>1</issue><spage>367</spage><epage>372</epage><pages>367-372</pages><issn>0885-7490</issn><eissn>1573-7365</eissn><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.</abstract><cop>New York</cop><pub>Springer US</pub><pmid>30392038</pmid><doi>10.1007/s11011-018-0334-z</doi><tpages>6</tpages></addata></record> |
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| subjects | Animal models Animals Biochemistry Biomedical and Life Sciences Biomedicine Biosynthesis Brain research Corpus striatum Corpus Striatum - pathology Degeneration Disease Models, Animal Dopamine receptors Echoes Encephalopathy Gene Silencing Genes Laboratory animals Leigh Disease - genetics Leigh Disease - pathology Medical treatment Medicine Metabolic Diseases Mice Mitochondria Molecular Chaperones - genetics Mutation Neostriatum Neurology Neurosciences Oncology Parkinson's disease Proteins Ribonucleic acid RNA Short Communication siRNA Substantia nigra Substantia Nigra - pathology |
| title | FOXRED1 silencing in mice: a possible animal model for Leigh syndrome |
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