Expression of cardiac α-actin spares extraocular muscles in skeletal muscle α-actin diseases – Quantification of striated α-actins by MRM-mass spectrometry
Abstract As with many skeletal muscle diseases, the extraocular muscles (EOMs) are spared in skeletal muscle α-actin diseases, with no ophthalmoplegia even in severely affected patients. We hypothesised that the extraocular muscles sparing in these patients was due to significant expression of cardi...
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| Veröffentlicht in: | Neuromuscular disorders : NMD 2008-12, Vol.18 (12), p.953-958 |
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| container_title | Neuromuscular disorders : NMD |
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| creator | Ravenscroft, Gianina Colley, Stephen M.J Walker, Kendall R Clement, Sophie Bringans, Scott Lipscombe, Richard Fabian, Victoria A Laing, Nigel G Nowak, Kristen J |
| description | Abstract As with many skeletal muscle diseases, the extraocular muscles (EOMs) are spared in skeletal muscle α-actin diseases, with no ophthalmoplegia even in severely affected patients. We hypothesised that the extraocular muscles sparing in these patients was due to significant expression of cardiac α-actin, the α-actin isoform expressed in heart and foetal skeletal muscle. We have shown by immunochemistry, Western blotting and a novel MRM-mass spectrometry technique, comparable levels of cardiac α-actin in the extraocular muscles of human, pig and sheep to those in the heart. The sparing of extraocular muscles in skeletal muscle α-actin disease is thus probably due to greater levels of cardiac α-actin, than the negligible amounts in skeletal muscles, diluting out the effects of the mutant skeletal muscle α-actin. |
| doi_str_mv | 10.1016/j.nmd.2008.09.010 |
| format | Article |
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We hypothesised that the extraocular muscles sparing in these patients was due to significant expression of cardiac α-actin, the α-actin isoform expressed in heart and foetal skeletal muscle. We have shown by immunochemistry, Western blotting and a novel MRM-mass spectrometry technique, comparable levels of cardiac α-actin in the extraocular muscles of human, pig and sheep to those in the heart. The sparing of extraocular muscles in skeletal muscle α-actin disease is thus probably due to greater levels of cardiac α-actin, than the negligible amounts in skeletal muscles, diluting out the effects of the mutant skeletal muscle α-actin.</description><identifier>ISSN: 0960-8966</identifier><identifier>EISSN: 1873-2364</identifier><identifier>DOI: 10.1016/j.nmd.2008.09.010</identifier><identifier>PMID: 18952430</identifier><language>eng</language><publisher>England: Elsevier B.V</publisher><subject>Actins - analysis ; Actins - metabolism ; Animals ; Blotting, Western ; Cardiac α-actin ; Extraocular muscle ; Guinea Pigs ; Humans ; Immunochemistry ; Mass Spectrometry ; MRM-mass spectrometry ; Muscle, Skeletal - metabolism ; Myocardium - metabolism ; Neurology ; Oculomotor Muscles - metabolism ; Protein Isoforms - analysis ; Protein Isoforms - metabolism ; Sheep ; Skeletal muscle α-actin ; Skeletal muscle α-actin myopathies ; Species Specificity</subject><ispartof>Neuromuscular disorders : NMD, 2008-12, Vol.18 (12), p.953-958</ispartof><rights>Elsevier B.V.</rights><rights>2008 Elsevier B.V.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c406t-e764eb1dc2b6ba6b5717f1e3e41ceac690f733e0e9baf9979f537e0c65687eb33</citedby><cites>FETCH-LOGICAL-c406t-e764eb1dc2b6ba6b5717f1e3e41ceac690f733e0e9baf9979f537e0c65687eb33</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.nmd.2008.09.010$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>315,781,785,3551,27926,27927,45997</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18952430$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ravenscroft, Gianina</creatorcontrib><creatorcontrib>Colley, Stephen M.J</creatorcontrib><creatorcontrib>Walker, Kendall R</creatorcontrib><creatorcontrib>Clement, Sophie</creatorcontrib><creatorcontrib>Bringans, Scott</creatorcontrib><creatorcontrib>Lipscombe, Richard</creatorcontrib><creatorcontrib>Fabian, Victoria A</creatorcontrib><creatorcontrib>Laing, Nigel G</creatorcontrib><creatorcontrib>Nowak, Kristen J</creatorcontrib><title>Expression of cardiac α-actin spares extraocular muscles in skeletal muscle α-actin diseases – Quantification of striated α-actins by MRM-mass spectrometry</title><title>Neuromuscular disorders : NMD</title><addtitle>Neuromuscul Disord</addtitle><description>Abstract As with many skeletal muscle diseases, the extraocular muscles (EOMs) are spared in skeletal muscle α-actin diseases, with no ophthalmoplegia even in severely affected patients. We hypothesised that the extraocular muscles sparing in these patients was due to significant expression of cardiac α-actin, the α-actin isoform expressed in heart and foetal skeletal muscle. We have shown by immunochemistry, Western blotting and a novel MRM-mass spectrometry technique, comparable levels of cardiac α-actin in the extraocular muscles of human, pig and sheep to those in the heart. The sparing of extraocular muscles in skeletal muscle α-actin disease is thus probably due to greater levels of cardiac α-actin, than the negligible amounts in skeletal muscles, diluting out the effects of the mutant skeletal muscle α-actin.</description><subject>Actins - analysis</subject><subject>Actins - metabolism</subject><subject>Animals</subject><subject>Blotting, Western</subject><subject>Cardiac α-actin</subject><subject>Extraocular muscle</subject><subject>Guinea Pigs</subject><subject>Humans</subject><subject>Immunochemistry</subject><subject>Mass Spectrometry</subject><subject>MRM-mass spectrometry</subject><subject>Muscle, Skeletal - metabolism</subject><subject>Myocardium - metabolism</subject><subject>Neurology</subject><subject>Oculomotor Muscles - metabolism</subject><subject>Protein Isoforms - analysis</subject><subject>Protein Isoforms - metabolism</subject><subject>Sheep</subject><subject>Skeletal muscle α-actin</subject><subject>Skeletal muscle α-actin myopathies</subject><subject>Species Specificity</subject><issn>0960-8966</issn><issn>1873-2364</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2008</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9UkuO1DAQtRCI6Rk4ABuUFbuEcpzYsZCQ0GhgkGaE-K0tx6lI7smncTloescduABX4CIcgpPgqFuMxIJVSVXvvVK9V4w94VBw4PL5tpjGrigBmgJ0ARzusQ1vlMhLIav7bANaQt5oKU_YKdEWgNdKqofshDe6LisBG_bj4nYXkMjPUzb3mbOh89Zlv37m1kU_ZbSzaZzhbQx2dstgQzYu5IbUW6c3OGC0w7F3R-s8oaUE-v3te_Z-sVP0vXc2HtdQDN5G7P4SKGv32fWH63y0RGkpuhjmEWPYP2IPejsQPj7WM_b59cWn88v86t2bt-evrnJXgYw5KllhyztXtrK1sq0VVz1HgRV3aJ3U0CshEFC3ttda6b4WCsHJWjYKWyHO2LOD7i7MXxakaEZPDofBTjgvZKRuqroUK5AfgC7MRAF7swt-tGFvOJg1FrM1KRazxmJAmxRL4jw9ii_tiN0d45hDArw4ADCd-NVjMOQ8Tg47H5IXppv9f-Vf_sN2g5-S38MN7pG28xKm5J3hhkoD5uP6F-tbQAMgayHFH7nluZo</recordid><startdate>20081201</startdate><enddate>20081201</enddate><creator>Ravenscroft, Gianina</creator><creator>Colley, Stephen M.J</creator><creator>Walker, Kendall R</creator><creator>Clement, Sophie</creator><creator>Bringans, Scott</creator><creator>Lipscombe, Richard</creator><creator>Fabian, Victoria A</creator><creator>Laing, Nigel G</creator><creator>Nowak, Kristen J</creator><general>Elsevier 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>7X8</scope></search><sort><creationdate>20081201</creationdate><title>Expression of cardiac α-actin spares extraocular muscles in skeletal muscle α-actin diseases – Quantification of striated α-actins by MRM-mass spectrometry</title><author>Ravenscroft, Gianina ; Colley, Stephen M.J ; Walker, Kendall R ; Clement, Sophie ; Bringans, Scott ; Lipscombe, Richard ; Fabian, Victoria A ; Laing, Nigel G ; Nowak, Kristen J</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c406t-e764eb1dc2b6ba6b5717f1e3e41ceac690f733e0e9baf9979f537e0c65687eb33</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2008</creationdate><topic>Actins - analysis</topic><topic>Actins - metabolism</topic><topic>Animals</topic><topic>Blotting, Western</topic><topic>Cardiac α-actin</topic><topic>Extraocular muscle</topic><topic>Guinea Pigs</topic><topic>Humans</topic><topic>Immunochemistry</topic><topic>Mass Spectrometry</topic><topic>MRM-mass spectrometry</topic><topic>Muscle, Skeletal - metabolism</topic><topic>Myocardium - metabolism</topic><topic>Neurology</topic><topic>Oculomotor Muscles - metabolism</topic><topic>Protein Isoforms - analysis</topic><topic>Protein Isoforms - metabolism</topic><topic>Sheep</topic><topic>Skeletal muscle α-actin</topic><topic>Skeletal muscle α-actin myopathies</topic><topic>Species Specificity</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ravenscroft, Gianina</creatorcontrib><creatorcontrib>Colley, Stephen M.J</creatorcontrib><creatorcontrib>Walker, Kendall R</creatorcontrib><creatorcontrib>Clement, Sophie</creatorcontrib><creatorcontrib>Bringans, Scott</creatorcontrib><creatorcontrib>Lipscombe, Richard</creatorcontrib><creatorcontrib>Fabian, Victoria A</creatorcontrib><creatorcontrib>Laing, Nigel G</creatorcontrib><creatorcontrib>Nowak, Kristen J</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Neuromuscular disorders : NMD</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ravenscroft, Gianina</au><au>Colley, Stephen M.J</au><au>Walker, Kendall R</au><au>Clement, Sophie</au><au>Bringans, Scott</au><au>Lipscombe, Richard</au><au>Fabian, Victoria A</au><au>Laing, Nigel G</au><au>Nowak, Kristen J</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Expression of cardiac α-actin spares extraocular muscles in skeletal muscle α-actin diseases – Quantification of striated α-actins by MRM-mass spectrometry</atitle><jtitle>Neuromuscular disorders : NMD</jtitle><addtitle>Neuromuscul Disord</addtitle><date>2008-12-01</date><risdate>2008</risdate><volume>18</volume><issue>12</issue><spage>953</spage><epage>958</epage><pages>953-958</pages><issn>0960-8966</issn><eissn>1873-2364</eissn><abstract>Abstract As with many skeletal muscle diseases, the extraocular muscles (EOMs) are spared in skeletal muscle α-actin diseases, with no ophthalmoplegia even in severely affected patients. We hypothesised that the extraocular muscles sparing in these patients was due to significant expression of cardiac α-actin, the α-actin isoform expressed in heart and foetal skeletal muscle. We have shown by immunochemistry, Western blotting and a novel MRM-mass spectrometry technique, comparable levels of cardiac α-actin in the extraocular muscles of human, pig and sheep to those in the heart. The sparing of extraocular muscles in skeletal muscle α-actin disease is thus probably due to greater levels of cardiac α-actin, than the negligible amounts in skeletal muscles, diluting out the effects of the mutant skeletal muscle α-actin.</abstract><cop>England</cop><pub>Elsevier B.V</pub><pmid>18952430</pmid><doi>10.1016/j.nmd.2008.09.010</doi><tpages>6</tpages></addata></record> |
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| subjects | Actins - analysis Actins - metabolism Animals Blotting, Western Cardiac α-actin Extraocular muscle Guinea Pigs Humans Immunochemistry Mass Spectrometry MRM-mass spectrometry Muscle, Skeletal - metabolism Myocardium - metabolism Neurology Oculomotor Muscles - metabolism Protein Isoforms - analysis Protein Isoforms - metabolism Sheep Skeletal muscle α-actin Skeletal muscle α-actin myopathies Species Specificity |
| title | Expression of cardiac α-actin spares extraocular muscles in skeletal muscle α-actin diseases – Quantification of striated α-actins by MRM-mass spectrometry |
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