Absence of T and B lymphocytes modulates dystrophic features in dysferlin deficient animal model
Dysferlin mutations cause muscular dystrophy (dysferlinopathy) characterized by adult onset muscle weakness, high serum creatine kinase levels, attenuation of muscle regeneration and a prominent inflammatory infiltrate. In order to verify the role of lymphocytes and immune cells on this disease, we...
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| Veröffentlicht in: | Experimental cell research 2012-06, Vol.318 (10), p.1160-1174 |
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| creator | Farini, Andrea Sitzia, Clementina Navarro, Claire D'Antona, Giuseppe Belicchi, Marzia Parolini, Daniele Del Fraro, Giulia Razini, Paola Bottinelli, Roberto Meregalli, Mirella Torrente, Yvan |
| description | Dysferlin mutations cause muscular dystrophy (dysferlinopathy) characterized by adult onset muscle weakness, high serum creatine kinase levels, attenuation of muscle regeneration and a prominent inflammatory infiltrate. In order to verify the role of lymphocytes and immune cells on this disease, we generated the Scid/A/J transgenic mice and compared these animals with the age-matched A/J mice. The absence of T and B lymphocytes in this animal model of dysferlinopathy resulted in an improvement of the muscle regeneration. Scid/A/J mice showed increased specific force in the myosin heavy chain 2A-expressing fibers of the diaphragm and abdominal muscles. Moreover, a partial reduction in complement deposition was observed together with a diminution in pro-inflammatory M1 macrophages. Consistent with this model, T and B lymphocytes seem to have a role in the muscle damaging immune response. The knowledge of the involvement of immune system in the development of dysferlinopathies could represent an important tool for their rescuing. By studying Scid/blAJ mice, we showed that it could be possible to modulate the pathological symptoms of these diseases by interfering with different components of the immune system. |
| doi_str_mv | 10.1016/j.yexcr.2012.03.010 |
| format | Article |
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In order to verify the role of lymphocytes and immune cells on this disease, we generated the Scid/A/J transgenic mice and compared these animals with the age-matched A/J mice. The absence of T and B lymphocytes in this animal model of dysferlinopathy resulted in an improvement of the muscle regeneration. Scid/A/J mice showed increased specific force in the myosin heavy chain 2A-expressing fibers of the diaphragm and abdominal muscles. Moreover, a partial reduction in complement deposition was observed together with a diminution in pro-inflammatory M1 macrophages. Consistent with this model, T and B lymphocytes seem to have a role in the muscle damaging immune response. The knowledge of the involvement of immune system in the development of dysferlinopathies could represent an important tool for their rescuing. By studying Scid/blAJ mice, we showed that it could be possible to modulate the pathological symptoms of these diseases by interfering with different components of the immune system.</description><identifier>ISSN: 0014-4827</identifier><identifier>EISSN: 1090-2422</identifier><identifier>DOI: 10.1016/j.yexcr.2012.03.010</identifier><identifier>PMID: 22465227</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Animals ; B-Lymphocytes - pathology ; Complement Membrane Attack Complex - metabolism ; Disease Models, Animal ; Dysferlin ; Dystrophin - metabolism ; Endothelial Cells - pathology ; Female ; Hybridization, Genetic ; Immune system ; In Vitro Techniques ; Inflammation ; Laminin - metabolism ; Lymphocytes ; Macrophages - pathology ; Male ; Membrane Proteins - deficiency ; Membrane Proteins - genetics ; Membrane Proteins - metabolism ; Mice ; Mice, Inbred C57BL ; Mice, Knockout ; Mice, SCID ; Muscle Contraction ; Muscle, Skeletal - blood supply ; Muscle, Skeletal - metabolism ; Muscle, Skeletal - pathology ; Muscle, Skeletal - physiopathology ; Muscular dystrophy ; Muscular Dystrophy, Animal - metabolism ; Muscular Dystrophy, Animal - pathology ; Muscular system ; Regeneration ; Rodents ; Sarcoglycans - metabolism ; Sarcolemma - genetics ; Sarcolemma - metabolism ; Sarcolemma - pathology ; Scid/A/J mice ; T-Lymphocytes - pathology</subject><ispartof>Experimental cell research, 2012-06, Vol.318 (10), p.1160-1174</ispartof><rights>2012 Elsevier Inc.</rights><rights>Copyright © 2012 Elsevier Inc. All rights reserved.</rights><rights>Copyright © 2012 Elsevier B.V. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c387t-d06edc4dd2ee18dfdbb64771a2aa57c33b499d3cc9f6d047a20d1aeaaa2cb9643</citedby><cites>FETCH-LOGICAL-c387t-d06edc4dd2ee18dfdbb64771a2aa57c33b499d3cc9f6d047a20d1aeaaa2cb9643</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.yexcr.2012.03.010$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22465227$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Farini, Andrea</creatorcontrib><creatorcontrib>Sitzia, Clementina</creatorcontrib><creatorcontrib>Navarro, Claire</creatorcontrib><creatorcontrib>D'Antona, Giuseppe</creatorcontrib><creatorcontrib>Belicchi, Marzia</creatorcontrib><creatorcontrib>Parolini, Daniele</creatorcontrib><creatorcontrib>Del Fraro, Giulia</creatorcontrib><creatorcontrib>Razini, Paola</creatorcontrib><creatorcontrib>Bottinelli, Roberto</creatorcontrib><creatorcontrib>Meregalli, Mirella</creatorcontrib><creatorcontrib>Torrente, Yvan</creatorcontrib><title>Absence of T and B lymphocytes modulates dystrophic features in dysferlin deficient animal model</title><title>Experimental cell research</title><addtitle>Exp Cell Res</addtitle><description>Dysferlin mutations cause muscular dystrophy (dysferlinopathy) characterized by adult onset muscle weakness, high serum creatine kinase levels, attenuation of muscle regeneration and a prominent inflammatory infiltrate. In order to verify the role of lymphocytes and immune cells on this disease, we generated the Scid/A/J transgenic mice and compared these animals with the age-matched A/J mice. The absence of T and B lymphocytes in this animal model of dysferlinopathy resulted in an improvement of the muscle regeneration. Scid/A/J mice showed increased specific force in the myosin heavy chain 2A-expressing fibers of the diaphragm and abdominal muscles. Moreover, a partial reduction in complement deposition was observed together with a diminution in pro-inflammatory M1 macrophages. Consistent with this model, T and B lymphocytes seem to have a role in the muscle damaging immune response. The knowledge of the involvement of immune system in the development of dysferlinopathies could represent an important tool for their rescuing. By studying Scid/blAJ mice, we showed that it could be possible to modulate the pathological symptoms of these diseases by interfering with different components of the immune system.</description><subject>Animals</subject><subject>B-Lymphocytes - pathology</subject><subject>Complement Membrane Attack Complex - metabolism</subject><subject>Disease Models, Animal</subject><subject>Dysferlin</subject><subject>Dystrophin - metabolism</subject><subject>Endothelial Cells - pathology</subject><subject>Female</subject><subject>Hybridization, Genetic</subject><subject>Immune system</subject><subject>In Vitro Techniques</subject><subject>Inflammation</subject><subject>Laminin - metabolism</subject><subject>Lymphocytes</subject><subject>Macrophages - pathology</subject><subject>Male</subject><subject>Membrane Proteins - deficiency</subject><subject>Membrane Proteins - genetics</subject><subject>Membrane Proteins - metabolism</subject><subject>Mice</subject><subject>Mice, Inbred C57BL</subject><subject>Mice, Knockout</subject><subject>Mice, SCID</subject><subject>Muscle Contraction</subject><subject>Muscle, Skeletal - blood supply</subject><subject>Muscle, Skeletal - metabolism</subject><subject>Muscle, Skeletal - pathology</subject><subject>Muscle, Skeletal - physiopathology</subject><subject>Muscular dystrophy</subject><subject>Muscular Dystrophy, Animal - metabolism</subject><subject>Muscular Dystrophy, Animal - pathology</subject><subject>Muscular system</subject><subject>Regeneration</subject><subject>Rodents</subject><subject>Sarcoglycans - metabolism</subject><subject>Sarcolemma - genetics</subject><subject>Sarcolemma - metabolism</subject><subject>Sarcolemma - pathology</subject><subject>Scid/A/J mice</subject><subject>T-Lymphocytes - pathology</subject><issn>0014-4827</issn><issn>1090-2422</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kUtP3TAQha2qVbk8fkElFKkbNgnjB3ayYEFRH0hI3cDaOPZE-CqJL3ZSkX-Pw6UsuuhqRqPvnLHPEPKFQkWByvNtteCzjRUDyirgFVD4QDYUGiiZYOwj2QBQUYqaqQNymNIWAOqays_kgDEhLxhTG_Jw1SYcLRahK-4KM7riW9Evw-4x2GXCVAzBzb1ZO7ekKYbdo7dFh2aaY575cR13GPu1w85bj-OUbfxg-lWL_TH51Jk-4clbPSL3P77fXf8qb3__vLm-ui0tr9VUOpDorHCOIdLada5tpVCKGmbMhbKct6JpHLe26aQDoQwDRw0aY5htGyn4ETnb--5ieJoxTXrwyWLfmxHDnHSOjNIG-Cv69R90G-Y45tetFCglFVeZ4nvKxpBSxE7vYv5WXDK0clJv9esB9HoADVxnbVadvnnP7YDuXfM38Qxc7gHMYfzxGHVaQ7PofEQ7aRf8fxe8AGBLmR8</recordid><startdate>20120610</startdate><enddate>20120610</enddate><creator>Farini, Andrea</creator><creator>Sitzia, Clementina</creator><creator>Navarro, Claire</creator><creator>D'Antona, Giuseppe</creator><creator>Belicchi, Marzia</creator><creator>Parolini, Daniele</creator><creator>Del Fraro, Giulia</creator><creator>Razini, Paola</creator><creator>Bottinelli, Roberto</creator><creator>Meregalli, Mirella</creator><creator>Torrente, Yvan</creator><general>Elsevier Inc</general><general>Elsevier BV</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>7TK</scope><scope>7TM</scope><scope>8FD</scope><scope>FR3</scope><scope>P64</scope><scope>RC3</scope><scope>7X8</scope></search><sort><creationdate>20120610</creationdate><title>Absence of T and B lymphocytes modulates dystrophic features in dysferlin deficient animal model</title><author>Farini, Andrea ; Sitzia, Clementina ; Navarro, Claire ; D'Antona, Giuseppe ; Belicchi, Marzia ; Parolini, Daniele ; Del Fraro, Giulia ; Razini, Paola ; Bottinelli, Roberto ; Meregalli, Mirella ; Torrente, Yvan</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c387t-d06edc4dd2ee18dfdbb64771a2aa57c33b499d3cc9f6d047a20d1aeaaa2cb9643</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2012</creationdate><topic>Animals</topic><topic>B-Lymphocytes - pathology</topic><topic>Complement Membrane Attack Complex - metabolism</topic><topic>Disease Models, Animal</topic><topic>Dysferlin</topic><topic>Dystrophin - metabolism</topic><topic>Endothelial Cells - pathology</topic><topic>Female</topic><topic>Hybridization, Genetic</topic><topic>Immune system</topic><topic>In Vitro Techniques</topic><topic>Inflammation</topic><topic>Laminin - metabolism</topic><topic>Lymphocytes</topic><topic>Macrophages - pathology</topic><topic>Male</topic><topic>Membrane Proteins - deficiency</topic><topic>Membrane Proteins - genetics</topic><topic>Membrane Proteins - metabolism</topic><topic>Mice</topic><topic>Mice, Inbred C57BL</topic><topic>Mice, Knockout</topic><topic>Mice, SCID</topic><topic>Muscle Contraction</topic><topic>Muscle, Skeletal - blood supply</topic><topic>Muscle, Skeletal - metabolism</topic><topic>Muscle, Skeletal - pathology</topic><topic>Muscle, Skeletal - physiopathology</topic><topic>Muscular dystrophy</topic><topic>Muscular Dystrophy, Animal - metabolism</topic><topic>Muscular Dystrophy, Animal - pathology</topic><topic>Muscular system</topic><topic>Regeneration</topic><topic>Rodents</topic><topic>Sarcoglycans - metabolism</topic><topic>Sarcolemma - genetics</topic><topic>Sarcolemma - metabolism</topic><topic>Sarcolemma - pathology</topic><topic>Scid/A/J mice</topic><topic>T-Lymphocytes - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Farini, Andrea</creatorcontrib><creatorcontrib>Sitzia, Clementina</creatorcontrib><creatorcontrib>Navarro, Claire</creatorcontrib><creatorcontrib>D'Antona, Giuseppe</creatorcontrib><creatorcontrib>Belicchi, Marzia</creatorcontrib><creatorcontrib>Parolini, Daniele</creatorcontrib><creatorcontrib>Del Fraro, Giulia</creatorcontrib><creatorcontrib>Razini, Paola</creatorcontrib><creatorcontrib>Bottinelli, Roberto</creatorcontrib><creatorcontrib>Meregalli, Mirella</creatorcontrib><creatorcontrib>Torrente, Yvan</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Neurosciences Abstracts</collection><collection>Nucleic Acids Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Experimental cell research</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Farini, Andrea</au><au>Sitzia, Clementina</au><au>Navarro, Claire</au><au>D'Antona, Giuseppe</au><au>Belicchi, Marzia</au><au>Parolini, Daniele</au><au>Del Fraro, Giulia</au><au>Razini, Paola</au><au>Bottinelli, Roberto</au><au>Meregalli, Mirella</au><au>Torrente, Yvan</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Absence of T and B lymphocytes modulates dystrophic features in dysferlin deficient animal model</atitle><jtitle>Experimental cell research</jtitle><addtitle>Exp Cell Res</addtitle><date>2012-06-10</date><risdate>2012</risdate><volume>318</volume><issue>10</issue><spage>1160</spage><epage>1174</epage><pages>1160-1174</pages><issn>0014-4827</issn><eissn>1090-2422</eissn><abstract>Dysferlin mutations cause muscular dystrophy (dysferlinopathy) characterized by adult onset muscle weakness, high serum creatine kinase levels, attenuation of muscle regeneration and a prominent inflammatory infiltrate. In order to verify the role of lymphocytes and immune cells on this disease, we generated the Scid/A/J transgenic mice and compared these animals with the age-matched A/J mice. The absence of T and B lymphocytes in this animal model of dysferlinopathy resulted in an improvement of the muscle regeneration. Scid/A/J mice showed increased specific force in the myosin heavy chain 2A-expressing fibers of the diaphragm and abdominal muscles. Moreover, a partial reduction in complement deposition was observed together with a diminution in pro-inflammatory M1 macrophages. Consistent with this model, T and B lymphocytes seem to have a role in the muscle damaging immune response. The knowledge of the involvement of immune system in the development of dysferlinopathies could represent an important tool for their rescuing. By studying Scid/blAJ mice, we showed that it could be possible to modulate the pathological symptoms of these diseases by interfering with different components of the immune system.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>22465227</pmid><doi>10.1016/j.yexcr.2012.03.010</doi><tpages>15</tpages></addata></record> |
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| subjects | Animals B-Lymphocytes - pathology Complement Membrane Attack Complex - metabolism Disease Models, Animal Dysferlin Dystrophin - metabolism Endothelial Cells - pathology Female Hybridization, Genetic Immune system In Vitro Techniques Inflammation Laminin - metabolism Lymphocytes Macrophages - pathology Male Membrane Proteins - deficiency Membrane Proteins - genetics Membrane Proteins - metabolism Mice Mice, Inbred C57BL Mice, Knockout Mice, SCID Muscle Contraction Muscle, Skeletal - blood supply Muscle, Skeletal - metabolism Muscle, Skeletal - pathology Muscle, Skeletal - physiopathology Muscular dystrophy Muscular Dystrophy, Animal - metabolism Muscular Dystrophy, Animal - pathology Muscular system Regeneration Rodents Sarcoglycans - metabolism Sarcolemma - genetics Sarcolemma - metabolism Sarcolemma - pathology Scid/A/J mice T-Lymphocytes - pathology |
| title | Absence of T and B lymphocytes modulates dystrophic features in dysferlin deficient animal model |
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