Down's syndrome: Abnormal neuromuscular junction in tongue of transgenic mice with elevated levels of human Cu/Zn-superoxide dismutase
To investigate the possible involvement of Cu/Zn-superoxide dismutase (CuZnSOD) gene dosage in the neuropathological symptoms of Down's syndrome, we analyzed the tongue muscle of transgenic mice that express elevated levels of human CuZnSOD. The tongue neuromuscular junctions (NMJ) in the trans...
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| Veröffentlicht in: | Cell 1988-09, Vol.54 (6), p.823-829 |
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| creator | Avraham, Karen B. Schickler, Michael Sapoznikov, Dan Yarom, Rena Groner, Yoram |
| description | To investigate the possible involvement of Cu/Zn-superoxide dismutase (CuZnSOD) gene dosage in the neuropathological symptoms of Down's syndrome, we analyzed the tongue muscle of transgenic mice that express elevated levels of human CuZnSOD. The tongue neuromuscular junctions (NMJ) in the transgenic animals exhibited significant pathological changes, namely, withdrawal and destruction of some terminal axons and the development of multiple small terminals. The ratio of terminal axon area to postsynaptic membrane decreased, and secondary folds were often complex and hyperplastic. The morphological changes in the transgenic NMJ were similar to those previously seen in muscles of aging mice and rats as well as in tongue muscle of patients with Down's syndrome. The findings suggest that CuZnSOD gene dosage is involved in the pathological abnormalities of tongue NMJ observed in Down's syndrome patients. |
| doi_str_mv | 10.1016/S0092-8674(88)91153-1 |
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The tongue neuromuscular junctions (NMJ) in the transgenic animals exhibited significant pathological changes, namely, withdrawal and destruction of some terminal axons and the development of multiple small terminals. The ratio of terminal axon area to postsynaptic membrane decreased, and secondary folds were often complex and hyperplastic. The morphological changes in the transgenic NMJ were similar to those previously seen in muscles of aging mice and rats as well as in tongue muscle of patients with Down's syndrome. The findings suggest that CuZnSOD gene dosage is involved in the pathological abnormalities of tongue NMJ observed in Down's syndrome patients.</description><identifier>ISSN: 0092-8674</identifier><identifier>EISSN: 1097-4172</identifier><identifier>DOI: 10.1016/S0092-8674(88)91153-1</identifier><identifier>PMID: 2970304</identifier><identifier>CODEN: CELLB5</identifier><language>eng</language><publisher>Cambridge, MA: Elsevier Inc</publisher><subject>Age Factors ; Animals ; Biological and medical sciences ; Biotechnology ; Down Syndrome - physiopathology ; Fundamental and applied biological sciences. Psychology ; Gene Expression Regulation ; Genetic engineering ; Genetic technics ; Methods. Procedures. Technologies ; Mice ; Mice, Transgenic ; Microscopy, Electron ; Neuromuscular Junction - ultrastructure ; Superoxide Dismutase - genetics ; Tongue - innervation ; Tongue - ultrastructure</subject><ispartof>Cell, 1988-09, Vol.54 (6), p.823-829</ispartof><rights>1988 Cell Press</rights><rights>1990 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c441t-29ad73c04217d9fec03d59e8f81ff3b025805236c4c36b6a45a73e0d4b755a993</citedby><cites>FETCH-LOGICAL-c441t-29ad73c04217d9fec03d59e8f81ff3b025805236c4c36b6a45a73e0d4b755a993</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/S0092-8674(88)91153-1$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=6872672$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/2970304$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Avraham, Karen B.</creatorcontrib><creatorcontrib>Schickler, Michael</creatorcontrib><creatorcontrib>Sapoznikov, Dan</creatorcontrib><creatorcontrib>Yarom, Rena</creatorcontrib><creatorcontrib>Groner, Yoram</creatorcontrib><title>Down's syndrome: Abnormal neuromuscular junction in tongue of transgenic mice with elevated levels of human Cu/Zn-superoxide dismutase</title><title>Cell</title><addtitle>Cell</addtitle><description>To investigate the possible involvement of Cu/Zn-superoxide dismutase (CuZnSOD) gene dosage in the neuropathological symptoms of Down's syndrome, we analyzed the tongue muscle of transgenic mice that express elevated levels of human CuZnSOD. The tongue neuromuscular junctions (NMJ) in the transgenic animals exhibited significant pathological changes, namely, withdrawal and destruction of some terminal axons and the development of multiple small terminals. The ratio of terminal axon area to postsynaptic membrane decreased, and secondary folds were often complex and hyperplastic. The morphological changes in the transgenic NMJ were similar to those previously seen in muscles of aging mice and rats as well as in tongue muscle of patients with Down's syndrome. The findings suggest that CuZnSOD gene dosage is involved in the pathological abnormalities of tongue NMJ observed in Down's syndrome patients.</description><subject>Age Factors</subject><subject>Animals</subject><subject>Biological and medical sciences</subject><subject>Biotechnology</subject><subject>Down Syndrome - physiopathology</subject><subject>Fundamental and applied biological sciences. Psychology</subject><subject>Gene Expression Regulation</subject><subject>Genetic engineering</subject><subject>Genetic technics</subject><subject>Methods. Procedures. Technologies</subject><subject>Mice</subject><subject>Mice, Transgenic</subject><subject>Microscopy, Electron</subject><subject>Neuromuscular Junction - ultrastructure</subject><subject>Superoxide Dismutase - genetics</subject><subject>Tongue - innervation</subject><subject>Tongue - ultrastructure</subject><issn>0092-8674</issn><issn>1097-4172</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1988</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkE1v1DAQhi0EKtvCT6jkA4JyCLVjO3a4oGr5lCpxAC5cLMeetK4Se-uPlv4BfjfZ7mqvnEaaed6Z0YPQKSXvKKHd-Q9C-rZRneRnSr3tKRWsoU_QipJeNpzK9ilaHZDn6DjnG0KIEkIcoaO2l4QRvkJ_P8b78Cbj_BBcijO8xxdDiGk2Ew5Ql07Ntk4m4ZsabPExYB9wieGqAo4jLsmEfAXBWzx7C_jel2sME9yZAg4vFaa85a7rbAJe1_Pfocl1Ayn-8Q6w83muxWR4gZ6NZsrwcl9P0K_Pn36uvzaX3798W19cNpZzWpq2N04yS3hLpetHsIQ50YMaFR1HNpBWKCJa1lluWTd0hgsjGRDHBymE6Xt2gl7v9m5SvK2Qi559tjBNJkCsWUvFFGOCL6DYgTbFnBOMepP8bNKDpkRv_etH_3orVyulH_1ruuRO9wfqMIM7pPbCl_mr_dxka6Zx8Wd9PmCdkm0n2wX7sMMWf3DnIelsPQQLziewRbvo__PIP6-KpBI</recordid><startdate>19880909</startdate><enddate>19880909</enddate><creator>Avraham, Karen B.</creator><creator>Schickler, Michael</creator><creator>Sapoznikov, Dan</creator><creator>Yarom, Rena</creator><creator>Groner, Yoram</creator><general>Elsevier Inc</general><general>Cell Press</general><scope>IQODW</scope><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>19880909</creationdate><title>Down's syndrome: Abnormal neuromuscular junction in tongue of transgenic mice with elevated levels of human Cu/Zn-superoxide dismutase</title><author>Avraham, Karen B. ; Schickler, Michael ; Sapoznikov, Dan ; Yarom, Rena ; Groner, Yoram</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c441t-29ad73c04217d9fec03d59e8f81ff3b025805236c4c36b6a45a73e0d4b755a993</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1988</creationdate><topic>Age Factors</topic><topic>Animals</topic><topic>Biological and medical sciences</topic><topic>Biotechnology</topic><topic>Down Syndrome - physiopathology</topic><topic>Fundamental and applied biological sciences. Psychology</topic><topic>Gene Expression Regulation</topic><topic>Genetic engineering</topic><topic>Genetic technics</topic><topic>Methods. Procedures. Technologies</topic><topic>Mice</topic><topic>Mice, Transgenic</topic><topic>Microscopy, Electron</topic><topic>Neuromuscular Junction - ultrastructure</topic><topic>Superoxide Dismutase - genetics</topic><topic>Tongue - innervation</topic><topic>Tongue - ultrastructure</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Avraham, Karen B.</creatorcontrib><creatorcontrib>Schickler, Michael</creatorcontrib><creatorcontrib>Sapoznikov, Dan</creatorcontrib><creatorcontrib>Yarom, Rena</creatorcontrib><creatorcontrib>Groner, Yoram</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>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Cell</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Avraham, Karen B.</au><au>Schickler, Michael</au><au>Sapoznikov, Dan</au><au>Yarom, Rena</au><au>Groner, Yoram</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Down's syndrome: Abnormal neuromuscular junction in tongue of transgenic mice with elevated levels of human Cu/Zn-superoxide dismutase</atitle><jtitle>Cell</jtitle><addtitle>Cell</addtitle><date>1988-09-09</date><risdate>1988</risdate><volume>54</volume><issue>6</issue><spage>823</spage><epage>829</epage><pages>823-829</pages><issn>0092-8674</issn><eissn>1097-4172</eissn><coden>CELLB5</coden><abstract>To investigate the possible involvement of Cu/Zn-superoxide dismutase (CuZnSOD) gene dosage in the neuropathological symptoms of Down's syndrome, we analyzed the tongue muscle of transgenic mice that express elevated levels of human CuZnSOD. The tongue neuromuscular junctions (NMJ) in the transgenic animals exhibited significant pathological changes, namely, withdrawal and destruction of some terminal axons and the development of multiple small terminals. The ratio of terminal axon area to postsynaptic membrane decreased, and secondary folds were often complex and hyperplastic. The morphological changes in the transgenic NMJ were similar to those previously seen in muscles of aging mice and rats as well as in tongue muscle of patients with Down's syndrome. The findings suggest that CuZnSOD gene dosage is involved in the pathological abnormalities of tongue NMJ observed in Down's syndrome patients.</abstract><cop>Cambridge, MA</cop><pub>Elsevier Inc</pub><pmid>2970304</pmid><doi>10.1016/S0092-8674(88)91153-1</doi><tpages>7</tpages></addata></record> |
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| ispartof | Cell, 1988-09, Vol.54 (6), p.823-829 |
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| subjects | Age Factors Animals Biological and medical sciences Biotechnology Down Syndrome - physiopathology Fundamental and applied biological sciences. Psychology Gene Expression Regulation Genetic engineering Genetic technics Methods. Procedures. Technologies Mice Mice, Transgenic Microscopy, Electron Neuromuscular Junction - ultrastructure Superoxide Dismutase - genetics Tongue - innervation Tongue - ultrastructure |
| title | Down's syndrome: Abnormal neuromuscular junction in tongue of transgenic mice with elevated levels of human Cu/Zn-superoxide dismutase |
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