Defects in nerve conduction velocity and different muscle fibre-type specificity contribute to muscle weakness in Ts1Cje Down syndrome mouse model
Down syndrome (DS) is a genetic disorder caused by presence of extra copy of human chromosome 21. It is characterised by several clinical phenotypes. Motor dysfunction due to hypotonia is commonly seen in individuals with DS and its etiology is yet unknown. Ts1Cje, which has a partial trisomy (Mmu16...
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| creator | Bala, Usman Leong, Melody Pui-Yee Lim, Chai Ling Shahar, Hayati Kadir Othman, Fauziah Lai, Mei-I Law, Zhe-Kang Ramli, Khairunnisa Htwe, Ohnmar Ling, King-Hwa Cheah, Pike-See |
| description | Down syndrome (DS) is a genetic disorder caused by presence of extra copy of human chromosome 21. It is characterised by several clinical phenotypes. Motor dysfunction due to hypotonia is commonly seen in individuals with DS and its etiology is yet unknown. Ts1Cje, which has a partial trisomy (Mmu16) homologous to Hsa21, is well reported to exhibit various typical neuropathological features seen in individuals with DS. This study investigated the role of skeletal muscles and peripheral nerve defects in contributing to muscle weakness in Ts1Cje mice.
Assessment of the motor performance showed that, the forelimb grip strength was significantly (P |
| doi_str_mv | 10.1371/journal.pone.0197711 |
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Assessment of the motor performance showed that, the forelimb grip strength was significantly (P<0.0001) greater in the WT mice compared to Ts1Cje mice regardless of gender. The average survival time of the WT mice during the hanging wire test was significantly (P<0.0001) greater compared to the Ts1Cje mice. Also, the WT mice performed significantly (P<0.05) better than the Ts1Cje mice in the latency to maintain a coordinated motor movement against the rotating rod. Adult Ts1Cje mice exhibited significantly (P<0.001) lower nerve conduction velocity compared with their aged matched WT mice. Further analysis showed a significantly (P<0.001) higher population of type I fibres in WT compared to Ts1Cje mice. Also, there was significantly (P<0.01) higher population of COX deficient fibres in Ts1Cje mice. Expression of Myf5 was significantly (P<0.05) reduced in triceps of Ts1Cje mice while MyoD expression was significantly (P<0.05) increased in quadriceps of Ts1Cje mice.
Ts1Cje mice exhibited weaker muscle strength. The lower population of the type I fibres and higher population of COX deficient fibres in Ts1Cje mice may contribute to the muscle weakness seen in this mouse model for DS.]]></description><identifier>ISSN: 1932-6203</identifier><identifier>EISSN: 1932-6203</identifier><identifier>DOI: 10.1371/journal.pone.0197711</identifier><identifier>PMID: 29795634</identifier><language>eng</language><publisher>United States: Public Library of Science</publisher><subject>Animals ; Biology and Life Sciences ; Care and treatment ; Chromosome 21 ; Chromosomes ; Complications and side effects ; Defects ; Development and progression ; Disease Models, Animal ; Down syndrome ; Down Syndrome - complications ; Down Syndrome - metabolism ; Down Syndrome - pathology ; Down's syndrome ; Electron Transport Complex IV - metabolism ; Etiology ; Female ; Fetuses ; Fibers ; Gene Expression Regulation ; Genetic disorders ; Genetics ; Genomics ; Genotype ; Grip strength ; Hand Strength - physiology ; Health sciences ; Homology ; Hypotonia ; Latency ; Male ; Medicine ; Medicine and Health Sciences ; Mice ; Motor ability ; Motor Activity - physiology ; Motor task performance ; Muscle Fibers, Skeletal - metabolism ; Muscle Fibers, Skeletal - pathology ; Muscle strength ; Muscle Weakness - complications ; Muscle Weakness - metabolism ; Muscle Weakness - pathology ; Muscle, Skeletal - metabolism ; Muscle, Skeletal - pathology ; Muscles ; Muscular strength ; Musculoskeletal diseases ; Musculoskeletal system ; MyoD protein ; MyoD Protein - metabolism ; Myogenic Regulatory Factor 5 - metabolism ; Nerve conduction ; Neural Conduction - physiology ; Phenotypes ; Quadriceps muscle ; Research and Analysis Methods ; Rodents ; Skeletal muscle ; Trisomy ; Velocity</subject><ispartof>PloS one, 2018-05, Vol.13 (5), p.e0197711-e0197711</ispartof><rights>COPYRIGHT 2018 Public Library of Science</rights><rights>2018 Bala et al. This is an open access article distributed under the terms of the Creative Commons Attribution License: http://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>2018 Bala et al 2018 Bala et al</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c692t-28e45c7a34a8f34c7ca364551a55c631b7cc2de913e33ea43b3b4d862804daa3</citedby><cites>FETCH-LOGICAL-c692t-28e45c7a34a8f34c7ca364551a55c631b7cc2de913e33ea43b3b4d862804daa3</cites><orcidid>0000-0002-4634-1252</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5967806/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC5967806/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,860,881,2096,2915,23845,27901,27902,53766,53768,79343,79344</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29795634$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Bala, Usman</creatorcontrib><creatorcontrib>Leong, Melody Pui-Yee</creatorcontrib><creatorcontrib>Lim, Chai Ling</creatorcontrib><creatorcontrib>Shahar, Hayati Kadir</creatorcontrib><creatorcontrib>Othman, Fauziah</creatorcontrib><creatorcontrib>Lai, Mei-I</creatorcontrib><creatorcontrib>Law, Zhe-Kang</creatorcontrib><creatorcontrib>Ramli, Khairunnisa</creatorcontrib><creatorcontrib>Htwe, Ohnmar</creatorcontrib><creatorcontrib>Ling, King-Hwa</creatorcontrib><creatorcontrib>Cheah, Pike-See</creatorcontrib><title>Defects in nerve conduction velocity and different muscle fibre-type specificity contribute to muscle weakness in Ts1Cje Down syndrome mouse model</title><title>PloS one</title><addtitle>PLoS One</addtitle><description><![CDATA[Down syndrome (DS) is a genetic disorder caused by presence of extra copy of human chromosome 21. It is characterised by several clinical phenotypes. Motor dysfunction due to hypotonia is commonly seen in individuals with DS and its etiology is yet unknown. Ts1Cje, which has a partial trisomy (Mmu16) homologous to Hsa21, is well reported to exhibit various typical neuropathological features seen in individuals with DS. This study investigated the role of skeletal muscles and peripheral nerve defects in contributing to muscle weakness in Ts1Cje mice.
Assessment of the motor performance showed that, the forelimb grip strength was significantly (P<0.0001) greater in the WT mice compared to Ts1Cje mice regardless of gender. The average survival time of the WT mice during the hanging wire test was significantly (P<0.0001) greater compared to the Ts1Cje mice. Also, the WT mice performed significantly (P<0.05) better than the Ts1Cje mice in the latency to maintain a coordinated motor movement against the rotating rod. Adult Ts1Cje mice exhibited significantly (P<0.001) lower nerve conduction velocity compared with their aged matched WT mice. Further analysis showed a significantly (P<0.001) higher population of type I fibres in WT compared to Ts1Cje mice. Also, there was significantly (P<0.01) higher population of COX deficient fibres in Ts1Cje mice. Expression of Myf5 was significantly (P<0.05) reduced in triceps of Ts1Cje mice while MyoD expression was significantly (P<0.05) increased in quadriceps of Ts1Cje mice.
Ts1Cje mice exhibited weaker muscle strength. The lower population of the type I fibres and higher population of COX deficient fibres in Ts1Cje mice may contribute to the muscle weakness seen in this mouse model for DS.]]></description><subject>Animals</subject><subject>Biology and Life Sciences</subject><subject>Care and treatment</subject><subject>Chromosome 21</subject><subject>Chromosomes</subject><subject>Complications and side effects</subject><subject>Defects</subject><subject>Development and progression</subject><subject>Disease Models, Animal</subject><subject>Down syndrome</subject><subject>Down Syndrome - complications</subject><subject>Down Syndrome - metabolism</subject><subject>Down Syndrome - pathology</subject><subject>Down's syndrome</subject><subject>Electron Transport Complex IV - metabolism</subject><subject>Etiology</subject><subject>Female</subject><subject>Fetuses</subject><subject>Fibers</subject><subject>Gene Expression Regulation</subject><subject>Genetic disorders</subject><subject>Genetics</subject><subject>Genomics</subject><subject>Genotype</subject><subject>Grip strength</subject><subject>Hand Strength - physiology</subject><subject>Health sciences</subject><subject>Homology</subject><subject>Hypotonia</subject><subject>Latency</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine and Health Sciences</subject><subject>Mice</subject><subject>Motor ability</subject><subject>Motor Activity - physiology</subject><subject>Motor task performance</subject><subject>Muscle Fibers, Skeletal - metabolism</subject><subject>Muscle Fibers, Skeletal - pathology</subject><subject>Muscle strength</subject><subject>Muscle Weakness - complications</subject><subject>Muscle Weakness - metabolism</subject><subject>Muscle Weakness - pathology</subject><subject>Muscle, Skeletal - metabolism</subject><subject>Muscle, Skeletal - pathology</subject><subject>Muscles</subject><subject>Muscular strength</subject><subject>Musculoskeletal diseases</subject><subject>Musculoskeletal system</subject><subject>MyoD protein</subject><subject>MyoD Protein - metabolism</subject><subject>Myogenic Regulatory Factor 5 - metabolism</subject><subject>Nerve conduction</subject><subject>Neural Conduction - physiology</subject><subject>Phenotypes</subject><subject>Quadriceps muscle</subject><subject>Research and Analysis Methods</subject><subject>Rodents</subject><subject>Skeletal muscle</subject><subject>Trisomy</subject><subject>Velocity</subject><issn>1932-6203</issn><issn>1932-6203</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><sourceid>DOA</sourceid><recordid>eNqNk99qFDEUxgdRbK2-gWhAEL3YNZlMkpkbobT-KRQKWrwNmeTMNutMsiaZ1n0Nn9jM7rZ0pRcykAnJ73wn50tOUbwkeE6oIB-WfgxO9fOVdzDHpBGCkEfFIWloOeMlpo_vzQ-KZzEuMWa05vxpcVA2omGcVofFn1PoQKeIrEMOwjUg7Z0ZdbLeoWvovbZpjZQzyNiugwAuoWGMugfU2TbALK1XgOIKtO3shs3xKdh2TICSv2VvQP10EDdpLiM5WQI69TcOxbUzwQ-ABj_GaTTQPy-edKqP8GL3PyouP3-6PPk6O7_4cnZyfD7TvCnTrKyhYlooWqm6o5UWWlFeMUYUY5pT0gqtSwMNoUApqIq2tK1MzcsaV0YpelS83squeh_lzs0oS1xRQUX2JxNnW8J4tZSrYAcV1tIrKzcLPiykCsnm-mRb1gprwwVmoqpN2RIwpsFcmNpAWddZ6-Mu29gOYHT2Mah-T3R_x9krufDXkjVc1JhngXc7geB_jRCTHGzU0PfKQfZuOjcreSMwyeibf9CHq9tRC5ULsK7zOa-eROUxqzBhjG_OPX-Ayp-Bwearhs7m9b2A93sB03OA32mhxhjl2fdv_89e_Nhn395jr0D16Sr6fpweatwHqy2og48xQHdnMsFy6pxbN-TUOXLXOTns1f0Lugu6bRX6F5_hFik</recordid><startdate>20180524</startdate><enddate>20180524</enddate><creator>Bala, Usman</creator><creator>Leong, Melody Pui-Yee</creator><creator>Lim, Chai Ling</creator><creator>Shahar, Hayati Kadir</creator><creator>Othman, Fauziah</creator><creator>Lai, Mei-I</creator><creator>Law, Zhe-Kang</creator><creator>Ramli, Khairunnisa</creator><creator>Htwe, Ohnmar</creator><creator>Ling, King-Hwa</creator><creator>Cheah, Pike-See</creator><general>Public Library of Science</general><general>Public Library of Science (PLoS)</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>IOV</scope><scope>ISR</scope><scope>3V.</scope><scope>7QG</scope><scope>7QL</scope><scope>7QO</scope><scope>7RV</scope><scope>7SN</scope><scope>7SS</scope><scope>7T5</scope><scope>7TG</scope><scope>7TM</scope><scope>7U9</scope><scope>7X2</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8C1</scope><scope>8FD</scope><scope>8FE</scope><scope>8FG</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABJCF</scope><scope>ABUWG</scope><scope>AEUYN</scope><scope>AFKRA</scope><scope>ARAPS</scope><scope>ATCPS</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BGLVJ</scope><scope>BHPHI</scope><scope>C1K</scope><scope>CCPQU</scope><scope>D1I</scope><scope>DWQXO</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>KB.</scope><scope>KB0</scope><scope>KL.</scope><scope>L6V</scope><scope>LK8</scope><scope>M0K</scope><scope>M0S</scope><scope>M1P</scope><scope>M7N</scope><scope>M7P</scope><scope>M7S</scope><scope>NAPCQ</scope><scope>P5Z</scope><scope>P62</scope><scope>P64</scope><scope>PATMY</scope><scope>PDBOC</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>PTHSS</scope><scope>PYCSY</scope><scope>RC3</scope><scope>7X8</scope><scope>5PM</scope><scope>DOA</scope><orcidid>https://orcid.org/0000-0002-4634-1252</orcidid></search><sort><creationdate>20180524</creationdate><title>Defects in nerve conduction velocity and different muscle fibre-type specificity contribute to muscle weakness in Ts1Cje Down syndrome mouse model</title><author>Bala, Usman ; Leong, Melody Pui-Yee ; Lim, Chai Ling ; Shahar, Hayati Kadir ; Othman, Fauziah ; Lai, Mei-I ; Law, Zhe-Kang ; Ramli, Khairunnisa ; Htwe, Ohnmar ; Ling, King-Hwa ; Cheah, Pike-See</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c692t-28e45c7a34a8f34c7ca364551a55c631b7cc2de913e33ea43b3b4d862804daa3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Animals</topic><topic>Biology and Life Sciences</topic><topic>Care and treatment</topic><topic>Chromosome 21</topic><topic>Chromosomes</topic><topic>Complications and side effects</topic><topic>Defects</topic><topic>Development and progression</topic><topic>Disease Models, Animal</topic><topic>Down syndrome</topic><topic>Down Syndrome - 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Academic</collection><collection>PubMed Central (Full Participant titles)</collection><collection>DOAJ Directory of Open Access Journals</collection><jtitle>PloS one</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Bala, Usman</au><au>Leong, Melody Pui-Yee</au><au>Lim, Chai Ling</au><au>Shahar, Hayati Kadir</au><au>Othman, Fauziah</au><au>Lai, Mei-I</au><au>Law, Zhe-Kang</au><au>Ramli, Khairunnisa</au><au>Htwe, Ohnmar</au><au>Ling, King-Hwa</au><au>Cheah, Pike-See</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Defects in nerve conduction velocity and different muscle fibre-type specificity contribute to muscle weakness in Ts1Cje Down syndrome mouse model</atitle><jtitle>PloS one</jtitle><addtitle>PLoS One</addtitle><date>2018-05-24</date><risdate>2018</risdate><volume>13</volume><issue>5</issue><spage>e0197711</spage><epage>e0197711</epage><pages>e0197711-e0197711</pages><issn>1932-6203</issn><eissn>1932-6203</eissn><abstract><![CDATA[Down syndrome (DS) is a genetic disorder caused by presence of extra copy of human chromosome 21. It is characterised by several clinical phenotypes. Motor dysfunction due to hypotonia is commonly seen in individuals with DS and its etiology is yet unknown. Ts1Cje, which has a partial trisomy (Mmu16) homologous to Hsa21, is well reported to exhibit various typical neuropathological features seen in individuals with DS. This study investigated the role of skeletal muscles and peripheral nerve defects in contributing to muscle weakness in Ts1Cje mice.
Assessment of the motor performance showed that, the forelimb grip strength was significantly (P<0.0001) greater in the WT mice compared to Ts1Cje mice regardless of gender. The average survival time of the WT mice during the hanging wire test was significantly (P<0.0001) greater compared to the Ts1Cje mice. Also, the WT mice performed significantly (P<0.05) better than the Ts1Cje mice in the latency to maintain a coordinated motor movement against the rotating rod. Adult Ts1Cje mice exhibited significantly (P<0.001) lower nerve conduction velocity compared with their aged matched WT mice. Further analysis showed a significantly (P<0.001) higher population of type I fibres in WT compared to Ts1Cje mice. Also, there was significantly (P<0.01) higher population of COX deficient fibres in Ts1Cje mice. Expression of Myf5 was significantly (P<0.05) reduced in triceps of Ts1Cje mice while MyoD expression was significantly (P<0.05) increased in quadriceps of Ts1Cje mice.
Ts1Cje mice exhibited weaker muscle strength. The lower population of the type I fibres and higher population of COX deficient fibres in Ts1Cje mice may contribute to the muscle weakness seen in this mouse model for DS.]]></abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>29795634</pmid><doi>10.1371/journal.pone.0197711</doi><tpages>e0197711</tpages><orcidid>https://orcid.org/0000-0002-4634-1252</orcidid><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 1932-6203 |
| ispartof | PloS one, 2018-05, Vol.13 (5), p.e0197711-e0197711 |
| issn | 1932-6203 1932-6203 |
| language | eng |
| recordid | cdi_plos_journals_2043737795 |
| source | MEDLINE; DOAJ Directory of Open Access Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; PubMed Central; Free Full-Text Journals in Chemistry; Public Library of Science (PLoS) |
| subjects | Animals Biology and Life Sciences Care and treatment Chromosome 21 Chromosomes Complications and side effects Defects Development and progression Disease Models, Animal Down syndrome Down Syndrome - complications Down Syndrome - metabolism Down Syndrome - pathology Down's syndrome Electron Transport Complex IV - metabolism Etiology Female Fetuses Fibers Gene Expression Regulation Genetic disorders Genetics Genomics Genotype Grip strength Hand Strength - physiology Health sciences Homology Hypotonia Latency Male Medicine Medicine and Health Sciences Mice Motor ability Motor Activity - physiology Motor task performance Muscle Fibers, Skeletal - metabolism Muscle Fibers, Skeletal - pathology Muscle strength Muscle Weakness - complications Muscle Weakness - metabolism Muscle Weakness - pathology Muscle, Skeletal - metabolism Muscle, Skeletal - pathology Muscles Muscular strength Musculoskeletal diseases Musculoskeletal system MyoD protein MyoD Protein - metabolism Myogenic Regulatory Factor 5 - metabolism Nerve conduction Neural Conduction - physiology Phenotypes Quadriceps muscle Research and Analysis Methods Rodents Skeletal muscle Trisomy Velocity |
| title | Defects in nerve conduction velocity and different muscle fibre-type specificity contribute to muscle weakness in Ts1Cje Down syndrome mouse model |
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