Type distribution of muscle fibres and their ultrastructure related to intracellular elemental composition as revealed by energy dispersive X-ray microanalysis: A study of multicore myopathy
Four cases of congenital myopathy, two children and two adults, are described whose light-microscopical and ultrastructural findings are consistent with a multicore myopathy. In all cases a hereditary etiology to the disorders is obvious presumably by an autosomal recessive trait. Fibres with centra...
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| Veröffentlicht in: | Journal of the neurological sciences 1986-11, Vol.76 (1), p.31-48 |
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| container_end_page | 48 |
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| container_title | Journal of the neurological sciences |
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| creator | Edström, Lars Mair, William G.P. Wroblewski, Romuald Hovmöller, Mia Malm, Gunilla |
| description | Four cases of congenital myopathy, two children and two adults, are described whose light-microscopical and ultrastructural findings are consistent with a multicore myopathy. In all cases a hereditary etiology to the disorders is obvious presumably by an autosomal recessive trait. Fibres with central nuclei and multiple minicores was a prominent finding in all cases.
Energy dispersive X-ray microanalysis of single muscle fibres revealed a normal intracellular content of elements (sodium, potassium and chlorine). This is in contrast to the findings in some cases of s.c. myotubular myopathy earlier described from our group where content of sodium and chlorine is markedly increased while that of potassium is decreased. It is suggested that in myotubular myopathy membrane dysfunction causing decreased ionic gradients is an important feature of the pathophysiology while in multicore myopathy other mechanisms, as a suggestion related to mitochondrial and myofibrillar function play a more prominent part.
From a diagnostic point of view it seems that X-ray microanalysis can be used to differentiate the two conditions. |
| doi_str_mv | 10.1016/0022-510X(86)90140-1 |
| format | Article |
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Energy dispersive X-ray microanalysis of single muscle fibres revealed a normal intracellular content of elements (sodium, potassium and chlorine). This is in contrast to the findings in some cases of s.c. myotubular myopathy earlier described from our group where content of sodium and chlorine is markedly increased while that of potassium is decreased. It is suggested that in myotubular myopathy membrane dysfunction causing decreased ionic gradients is an important feature of the pathophysiology while in multicore myopathy other mechanisms, as a suggestion related to mitochondrial and myofibrillar function play a more prominent part.
From a diagnostic point of view it seems that X-ray microanalysis can be used to differentiate the two conditions.</description><identifier>ISSN: 0022-510X</identifier><identifier>EISSN: 1878-5883</identifier><identifier>DOI: 10.1016/0022-510X(86)90140-1</identifier><identifier>PMID: 3783187</identifier><identifier>CODEN: JNSCAG</identifier><language>eng</language><publisher>Shannon: Elsevier B.V</publisher><subject>Adult ; Biological and medical sciences ; Child, Preschool ; Chlorine - analysis ; Congenital myopathy ; Core fibres ; Diseases of striated muscles. Neuromuscular diseases ; Electron Probe Microanalysis ; Female ; Humans ; ions ; man ; Medical sciences ; Microscopy, Electron ; Muscle ultrastructure ; muscles ; Muscles - analysis ; Muscles - ultrastructure ; Muscular Diseases - congenital ; Muscular Diseases - metabolism ; Muscular Diseases - pathology ; myopathy ; Neurology ; plasma membranes ; Potassium - analysis ; Sodium - analysis ; X-ray micro-analysis</subject><ispartof>Journal of the neurological sciences, 1986-11, Vol.76 (1), p.31-48</ispartof><rights>1986</rights><rights>1987 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/0022510X86901401$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,776,780,3536,27903,27904,65309</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=7915240$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/3783187$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Edström, Lars</creatorcontrib><creatorcontrib>Mair, William G.P.</creatorcontrib><creatorcontrib>Wroblewski, Romuald</creatorcontrib><creatorcontrib>Hovmöller, Mia</creatorcontrib><creatorcontrib>Malm, Gunilla</creatorcontrib><title>Type distribution of muscle fibres and their ultrastructure related to intracellular elemental composition as revealed by energy dispersive X-ray microanalysis: A study of multicore myopathy</title><title>Journal of the neurological sciences</title><addtitle>J Neurol Sci</addtitle><description>Four cases of congenital myopathy, two children and two adults, are described whose light-microscopical and ultrastructural findings are consistent with a multicore myopathy. In all cases a hereditary etiology to the disorders is obvious presumably by an autosomal recessive trait. Fibres with central nuclei and multiple minicores was a prominent finding in all cases.
Energy dispersive X-ray microanalysis of single muscle fibres revealed a normal intracellular content of elements (sodium, potassium and chlorine). This is in contrast to the findings in some cases of s.c. myotubular myopathy earlier described from our group where content of sodium and chlorine is markedly increased while that of potassium is decreased. It is suggested that in myotubular myopathy membrane dysfunction causing decreased ionic gradients is an important feature of the pathophysiology while in multicore myopathy other mechanisms, as a suggestion related to mitochondrial and myofibrillar function play a more prominent part.
From a diagnostic point of view it seems that X-ray microanalysis can be used to differentiate the two conditions.</description><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Child, Preschool</subject><subject>Chlorine - analysis</subject><subject>Congenital myopathy</subject><subject>Core fibres</subject><subject>Diseases of striated muscles. Neuromuscular diseases</subject><subject>Electron Probe Microanalysis</subject><subject>Female</subject><subject>Humans</subject><subject>ions</subject><subject>man</subject><subject>Medical sciences</subject><subject>Microscopy, Electron</subject><subject>Muscle ultrastructure</subject><subject>muscles</subject><subject>Muscles - analysis</subject><subject>Muscles - ultrastructure</subject><subject>Muscular Diseases - congenital</subject><subject>Muscular Diseases - metabolism</subject><subject>Muscular Diseases - pathology</subject><subject>myopathy</subject><subject>Neurology</subject><subject>plasma membranes</subject><subject>Potassium - analysis</subject><subject>Sodium - analysis</subject><subject>X-ray micro-analysis</subject><issn>0022-510X</issn><issn>1878-5883</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1986</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo9ks1q3DAQgEVpSTdp36AFHUpJD24ly7LlHAoh9A8CvaSQmxhL40ZFtlxJXvDL9dmqzS456fB986cZQt5w9pEz3n5irK4rydn9pWo_9Iw3rOLPyI6rTlVSKfGc7J6Ul-Q8pT-MsVap_oyciU6JIu7Iv7ttQWpdytENa3ZhpmGk05qMRzq6IWKiMFuaH9BFuvocoairyWtEGtFDxgIDdXMhBr1fPUSKHiecM3hqwrSE5B4TQyoRewRfQoaN4ozx93aovWBMbo_0voqw0cmZGGAGvyWXrug1TXm127Etn50JpfK0hQXyw_aKvBjBJ3x9ei_Ir69f7m6-V7c_v_24ub6tUNQ8VxZt3RgmhGSorGms6Ntu6OQ4ggQ79lB3dUEtNEPfdEaAVFJ0dVt3Bm3PjLgg7495lxj-rpiynlw6jAszhjVp3shGNJ0s4tuTuA4TWr1EN0Hc9OnDC3934pAM-DHCbFx60rqey7phRft81LAMtXcYdTIO59KNi2iytsFpzvThDvRhyfqwZK1a_XgHmov_OCmqRA</recordid><startdate>19861101</startdate><enddate>19861101</enddate><creator>Edström, Lars</creator><creator>Mair, William G.P.</creator><creator>Wroblewski, Romuald</creator><creator>Hovmöller, Mia</creator><creator>Malm, Gunilla</creator><general>Elsevier B.V</general><general>Elsevier Science</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>8FD</scope><scope>FR3</scope><scope>M7Z</scope><scope>P64</scope></search><sort><creationdate>19861101</creationdate><title>Type distribution of muscle fibres and their ultrastructure related to intracellular elemental composition as revealed by energy dispersive X-ray microanalysis: A study of multicore myopathy</title><author>Edström, Lars ; Mair, William G.P. ; Wroblewski, Romuald ; Hovmöller, Mia ; Malm, Gunilla</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-e321t-ded24c03350e8dc4d3967b75ffa5adf9a27250e6a4b947c3a585372627ced90c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1986</creationdate><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>Child, Preschool</topic><topic>Chlorine - analysis</topic><topic>Congenital myopathy</topic><topic>Core fibres</topic><topic>Diseases of striated muscles. Neuromuscular diseases</topic><topic>Electron Probe Microanalysis</topic><topic>Female</topic><topic>Humans</topic><topic>ions</topic><topic>man</topic><topic>Medical sciences</topic><topic>Microscopy, Electron</topic><topic>Muscle ultrastructure</topic><topic>muscles</topic><topic>Muscles - analysis</topic><topic>Muscles - ultrastructure</topic><topic>Muscular Diseases - congenital</topic><topic>Muscular Diseases - metabolism</topic><topic>Muscular Diseases - pathology</topic><topic>myopathy</topic><topic>Neurology</topic><topic>plasma membranes</topic><topic>Potassium - analysis</topic><topic>Sodium - analysis</topic><topic>X-ray micro-analysis</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Edström, Lars</creatorcontrib><creatorcontrib>Mair, William G.P.</creatorcontrib><creatorcontrib>Wroblewski, Romuald</creatorcontrib><creatorcontrib>Hovmöller, Mia</creatorcontrib><creatorcontrib>Malm, Gunilla</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>Biochemistry Abstracts 1</collection><collection>Biotechnology and BioEngineering Abstracts</collection><jtitle>Journal of the neurological sciences</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Edström, Lars</au><au>Mair, William G.P.</au><au>Wroblewski, Romuald</au><au>Hovmöller, Mia</au><au>Malm, Gunilla</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Type distribution of muscle fibres and their ultrastructure related to intracellular elemental composition as revealed by energy dispersive X-ray microanalysis: A study of multicore myopathy</atitle><jtitle>Journal of the neurological sciences</jtitle><addtitle>J Neurol Sci</addtitle><date>1986-11-01</date><risdate>1986</risdate><volume>76</volume><issue>1</issue><spage>31</spage><epage>48</epage><pages>31-48</pages><issn>0022-510X</issn><eissn>1878-5883</eissn><coden>JNSCAG</coden><abstract>Four cases of congenital myopathy, two children and two adults, are described whose light-microscopical and ultrastructural findings are consistent with a multicore myopathy. In all cases a hereditary etiology to the disorders is obvious presumably by an autosomal recessive trait. Fibres with central nuclei and multiple minicores was a prominent finding in all cases.
Energy dispersive X-ray microanalysis of single muscle fibres revealed a normal intracellular content of elements (sodium, potassium and chlorine). This is in contrast to the findings in some cases of s.c. myotubular myopathy earlier described from our group where content of sodium and chlorine is markedly increased while that of potassium is decreased. It is suggested that in myotubular myopathy membrane dysfunction causing decreased ionic gradients is an important feature of the pathophysiology while in multicore myopathy other mechanisms, as a suggestion related to mitochondrial and myofibrillar function play a more prominent part.
From a diagnostic point of view it seems that X-ray microanalysis can be used to differentiate the two conditions.</abstract><cop>Shannon</cop><pub>Elsevier B.V</pub><pmid>3783187</pmid><doi>10.1016/0022-510X(86)90140-1</doi><tpages>18</tpages></addata></record> |
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| subjects | Adult Biological and medical sciences Child, Preschool Chlorine - analysis Congenital myopathy Core fibres Diseases of striated muscles. Neuromuscular diseases Electron Probe Microanalysis Female Humans ions man Medical sciences Microscopy, Electron Muscle ultrastructure muscles Muscles - analysis Muscles - ultrastructure Muscular Diseases - congenital Muscular Diseases - metabolism Muscular Diseases - pathology myopathy Neurology plasma membranes Potassium - analysis Sodium - analysis X-ray micro-analysis |
| title | Type distribution of muscle fibres and their ultrastructure related to intracellular elemental composition as revealed by energy dispersive X-ray microanalysis: A study of multicore myopathy |
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