Normal nerve striations are altered in the trembler-J mouse, a model of charcot-marie-tooth disease
ABSTRACT Introduction: This study was initiated because it was noted that the peripheral nerves of Trembler‐J mice (a model of human Charcot–Marie–Tooth disease) appear to lack normal striations. Methods: We performed confocal microscopy of whole sciatic nerves and tested the effect of axial stress...
Gespeichert in:
| Veröffentlicht in: | Muscle & nerve 2015-02, Vol.51 (2), p.246-252 |
|---|---|
| Hauptverfasser: | , , , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 252 |
|---|---|
| container_issue | 2 |
| container_start_page | 246 |
| container_title | Muscle & nerve |
| container_volume | 51 |
| creator | Power, Bridget J. O'reilly, Gavin Murphy, Robert Murphy, Keith J. Pickering, Mark Jones, James F.X. |
| description | ABSTRACT
Introduction: This study was initiated because it was noted that the peripheral nerves of Trembler‐J mice (a model of human Charcot–Marie–Tooth disease) appear to lack normal striations. Methods: We performed confocal microscopy of whole sciatic nerves and tested the effect of axial stress on impulse conduction. Results: We found that the axons of mutant mice were longer than those of the wild‐type (1.55 mm of axon/mm length of nerve vs. 1.28 mm/mm respectively). This axonal elongation altered the helical nerve striations (bands of Fontana). As nerves were stretched axially, the conduction distance became correspondingly shorter. The effect on latency was significantly greater in the more coiled nerves of Trembler‐J mice (P = 0.038). Conclusions: The finding that mice with a mutated peripheral myelin protein 22 (PMP22) possess excessively long axons may be related to the excess Schwann cell numbers found in this disorder. Muscle Nerve 51: 246–252, 2015 |
| doi_str_mv | 10.1002/mus.24303 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_1753498540</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>1753498540</sourcerecordid><originalsourceid>FETCH-LOGICAL-c4243-4d64cf0b4da5c62c932b1e8052e831cf14407202ad0d4a03128b344b76b1a9423</originalsourceid><addsrcrecordid>eNqFkc1u1TAQhS0EopfCghdAltiAhFv_TBJniSIooHJZQEV3luNMdF2SuLUdoG-P4bZdICFWMyN9czRzDiFPBT8SnMvjeU1HEhRX98hG8LZhULX6PtlwAZrVqj0_II9SuuCcC103D8mBBN2WodoQtw1xthNdMH5HmnL0NvuwJGojUjtljDhQv9C8Q5ojzv2EkX2gc1gTvqK2NANONIzU7Wx0IbPZRo8sh5B3dPAJbcLH5MFop4RPbuohOXv75kv3jp1-OnnfvT5lDsr1DIYa3Mh7GGzlaulaJXuBmlcStRJuFAC8kVzagQ9guRJS9wqgb-pe2BakOiQv9rqXMVytmLKZfXI4TXbBcq8RTaWg1RXw_6N1JZVWDaiCPv8LvQhrXMojhQIQUAyHQr3cUy6GlCKO5jL64sW1Edz8DsmUkMyfkAr77EZx7Wcc7sjbVApwvAd--Amv_61kPp59vpVk-w2fMv6827Dxm6kb1VTm6_bEdF3dbrfnnanUL_zhqIA</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1644140974</pqid></control><display><type>article</type><title>Normal nerve striations are altered in the trembler-J mouse, a model of charcot-marie-tooth disease</title><source>MEDLINE</source><source>Wiley Online Library Journals Frontfile Complete</source><creator>Power, Bridget J. ; O'reilly, Gavin ; Murphy, Robert ; Murphy, Keith J. ; Pickering, Mark ; Jones, James F.X.</creator><creatorcontrib>Power, Bridget J. ; O'reilly, Gavin ; Murphy, Robert ; Murphy, Keith J. ; Pickering, Mark ; Jones, James F.X.</creatorcontrib><description>ABSTRACT
Introduction: This study was initiated because it was noted that the peripheral nerves of Trembler‐J mice (a model of human Charcot–Marie–Tooth disease) appear to lack normal striations. Methods: We performed confocal microscopy of whole sciatic nerves and tested the effect of axial stress on impulse conduction. Results: We found that the axons of mutant mice were longer than those of the wild‐type (1.55 mm of axon/mm length of nerve vs. 1.28 mm/mm respectively). This axonal elongation altered the helical nerve striations (bands of Fontana). As nerves were stretched axially, the conduction distance became correspondingly shorter. The effect on latency was significantly greater in the more coiled nerves of Trembler‐J mice (P = 0.038). Conclusions: The finding that mice with a mutated peripheral myelin protein 22 (PMP22) possess excessively long axons may be related to the excess Schwann cell numbers found in this disorder. Muscle Nerve 51: 246–252, 2015</description><identifier>ISSN: 0148-639X</identifier><identifier>EISSN: 1097-4598</identifier><identifier>DOI: 10.1002/mus.24303</identifier><identifier>PMID: 24890015</identifier><identifier>CODEN: MUNEDE</identifier><language>eng</language><publisher>United States: Blackwell Publishing Ltd</publisher><subject>Action Potentials - physiology ; Animals ; Axons - pathology ; bands of Fontana ; Charcot-Marie-Tooth disease ; Charcot-Marie-Tooth Disease - genetics ; Charcot-Marie-Tooth Disease - metabolism ; Charcot-Marie-Tooth Disease - pathology ; Disease Models, Animal ; Electrophysiology ; Male ; Medical research ; Mice ; Mice, Inbred C57BL ; Mice, Neurologic Mutants ; Microscopy, Confocal ; Mutation - genetics ; Myelin Proteins - genetics ; Myelin Sheath - metabolism ; Myelin Sheath - pathology ; nerve stretch ; Neural Conduction - genetics ; Neural Conduction - physiology ; rat ; Rodents ; sciatic nerve ; Sciatic Nerve - metabolism ; Sciatic Nerve - physiopathology ; Stress, Mechanical</subject><ispartof>Muscle & nerve, 2015-02, Vol.51 (2), p.246-252</ispartof><rights>2014 Wiley Periodicals, Inc.</rights><rights>2015 Wiley Periodicals, Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4243-4d64cf0b4da5c62c932b1e8052e831cf14407202ad0d4a03128b344b76b1a9423</citedby><cites>FETCH-LOGICAL-c4243-4d64cf0b4da5c62c932b1e8052e831cf14407202ad0d4a03128b344b76b1a9423</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fmus.24303$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fmus.24303$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/24890015$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Power, Bridget J.</creatorcontrib><creatorcontrib>O'reilly, Gavin</creatorcontrib><creatorcontrib>Murphy, Robert</creatorcontrib><creatorcontrib>Murphy, Keith J.</creatorcontrib><creatorcontrib>Pickering, Mark</creatorcontrib><creatorcontrib>Jones, James F.X.</creatorcontrib><title>Normal nerve striations are altered in the trembler-J mouse, a model of charcot-marie-tooth disease</title><title>Muscle & nerve</title><addtitle>Muscle Nerve</addtitle><description>ABSTRACT
Introduction: This study was initiated because it was noted that the peripheral nerves of Trembler‐J mice (a model of human Charcot–Marie–Tooth disease) appear to lack normal striations. Methods: We performed confocal microscopy of whole sciatic nerves and tested the effect of axial stress on impulse conduction. Results: We found that the axons of mutant mice were longer than those of the wild‐type (1.55 mm of axon/mm length of nerve vs. 1.28 mm/mm respectively). This axonal elongation altered the helical nerve striations (bands of Fontana). As nerves were stretched axially, the conduction distance became correspondingly shorter. The effect on latency was significantly greater in the more coiled nerves of Trembler‐J mice (P = 0.038). Conclusions: The finding that mice with a mutated peripheral myelin protein 22 (PMP22) possess excessively long axons may be related to the excess Schwann cell numbers found in this disorder. Muscle Nerve 51: 246–252, 2015</description><subject>Action Potentials - physiology</subject><subject>Animals</subject><subject>Axons - pathology</subject><subject>bands of Fontana</subject><subject>Charcot-Marie-Tooth disease</subject><subject>Charcot-Marie-Tooth Disease - genetics</subject><subject>Charcot-Marie-Tooth Disease - metabolism</subject><subject>Charcot-Marie-Tooth Disease - pathology</subject><subject>Disease Models, Animal</subject><subject>Electrophysiology</subject><subject>Male</subject><subject>Medical research</subject><subject>Mice</subject><subject>Mice, Inbred C57BL</subject><subject>Mice, Neurologic Mutants</subject><subject>Microscopy, Confocal</subject><subject>Mutation - genetics</subject><subject>Myelin Proteins - genetics</subject><subject>Myelin Sheath - metabolism</subject><subject>Myelin Sheath - pathology</subject><subject>nerve stretch</subject><subject>Neural Conduction - genetics</subject><subject>Neural Conduction - physiology</subject><subject>rat</subject><subject>Rodents</subject><subject>sciatic nerve</subject><subject>Sciatic Nerve - metabolism</subject><subject>Sciatic Nerve - physiopathology</subject><subject>Stress, Mechanical</subject><issn>0148-639X</issn><issn>1097-4598</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkc1u1TAQhS0EopfCghdAltiAhFv_TBJniSIooHJZQEV3luNMdF2SuLUdoG-P4bZdICFWMyN9czRzDiFPBT8SnMvjeU1HEhRX98hG8LZhULX6PtlwAZrVqj0_II9SuuCcC103D8mBBN2WodoQtw1xthNdMH5HmnL0NvuwJGojUjtljDhQv9C8Q5ojzv2EkX2gc1gTvqK2NANONIzU7Wx0IbPZRo8sh5B3dPAJbcLH5MFop4RPbuohOXv75kv3jp1-OnnfvT5lDsr1DIYa3Mh7GGzlaulaJXuBmlcStRJuFAC8kVzagQ9guRJS9wqgb-pe2BakOiQv9rqXMVytmLKZfXI4TXbBcq8RTaWg1RXw_6N1JZVWDaiCPv8LvQhrXMojhQIQUAyHQr3cUy6GlCKO5jL64sW1Edz8DsmUkMyfkAr77EZx7Wcc7sjbVApwvAd--Amv_61kPp59vpVk-w2fMv6827Dxm6kb1VTm6_bEdF3dbrfnnanUL_zhqIA</recordid><startdate>201502</startdate><enddate>201502</enddate><creator>Power, Bridget J.</creator><creator>O'reilly, Gavin</creator><creator>Murphy, Robert</creator><creator>Murphy, Keith J.</creator><creator>Pickering, Mark</creator><creator>Jones, James F.X.</creator><general>Blackwell Publishing Ltd</general><general>Wiley Subscription Services, Inc</general><scope>BSCLL</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>7T5</scope><scope>7TK</scope><scope>7TM</scope><scope>7TS</scope><scope>7U7</scope><scope>7U9</scope><scope>C1K</scope><scope>H94</scope><scope>K9.</scope><scope>NAPCQ</scope><scope>7X8</scope></search><sort><creationdate>201502</creationdate><title>Normal nerve striations are altered in the trembler-J mouse, a model of charcot-marie-tooth disease</title><author>Power, Bridget J. ; O'reilly, Gavin ; Murphy, Robert ; Murphy, Keith J. ; Pickering, Mark ; Jones, James F.X.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4243-4d64cf0b4da5c62c932b1e8052e831cf14407202ad0d4a03128b344b76b1a9423</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><topic>Action Potentials - physiology</topic><topic>Animals</topic><topic>Axons - pathology</topic><topic>bands of Fontana</topic><topic>Charcot-Marie-Tooth disease</topic><topic>Charcot-Marie-Tooth Disease - genetics</topic><topic>Charcot-Marie-Tooth Disease - metabolism</topic><topic>Charcot-Marie-Tooth Disease - pathology</topic><topic>Disease Models, Animal</topic><topic>Electrophysiology</topic><topic>Male</topic><topic>Medical research</topic><topic>Mice</topic><topic>Mice, Inbred C57BL</topic><topic>Mice, Neurologic Mutants</topic><topic>Microscopy, Confocal</topic><topic>Mutation - genetics</topic><topic>Myelin Proteins - genetics</topic><topic>Myelin Sheath - metabolism</topic><topic>Myelin Sheath - pathology</topic><topic>nerve stretch</topic><topic>Neural Conduction - genetics</topic><topic>Neural Conduction - physiology</topic><topic>rat</topic><topic>Rodents</topic><topic>sciatic nerve</topic><topic>Sciatic Nerve - metabolism</topic><topic>Sciatic Nerve - physiopathology</topic><topic>Stress, Mechanical</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Power, Bridget J.</creatorcontrib><creatorcontrib>O'reilly, Gavin</creatorcontrib><creatorcontrib>Murphy, Robert</creatorcontrib><creatorcontrib>Murphy, Keith J.</creatorcontrib><creatorcontrib>Pickering, Mark</creatorcontrib><creatorcontrib>Jones, James F.X.</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Nucleic Acids Abstracts</collection><collection>Physical Education Index</collection><collection>Toxicology Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Environmental Sciences and Pollution Management</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Premium</collection><collection>MEDLINE - Academic</collection><jtitle>Muscle & nerve</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Power, Bridget J.</au><au>O'reilly, Gavin</au><au>Murphy, Robert</au><au>Murphy, Keith J.</au><au>Pickering, Mark</au><au>Jones, James F.X.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Normal nerve striations are altered in the trembler-J mouse, a model of charcot-marie-tooth disease</atitle><jtitle>Muscle & nerve</jtitle><addtitle>Muscle Nerve</addtitle><date>2015-02</date><risdate>2015</risdate><volume>51</volume><issue>2</issue><spage>246</spage><epage>252</epage><pages>246-252</pages><issn>0148-639X</issn><eissn>1097-4598</eissn><coden>MUNEDE</coden><abstract>ABSTRACT
Introduction: This study was initiated because it was noted that the peripheral nerves of Trembler‐J mice (a model of human Charcot–Marie–Tooth disease) appear to lack normal striations. Methods: We performed confocal microscopy of whole sciatic nerves and tested the effect of axial stress on impulse conduction. Results: We found that the axons of mutant mice were longer than those of the wild‐type (1.55 mm of axon/mm length of nerve vs. 1.28 mm/mm respectively). This axonal elongation altered the helical nerve striations (bands of Fontana). As nerves were stretched axially, the conduction distance became correspondingly shorter. The effect on latency was significantly greater in the more coiled nerves of Trembler‐J mice (P = 0.038). Conclusions: The finding that mice with a mutated peripheral myelin protein 22 (PMP22) possess excessively long axons may be related to the excess Schwann cell numbers found in this disorder. Muscle Nerve 51: 246–252, 2015</abstract><cop>United States</cop><pub>Blackwell Publishing Ltd</pub><pmid>24890015</pmid><doi>10.1002/mus.24303</doi><tpages>7</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0148-639X |
| ispartof | Muscle & nerve, 2015-02, Vol.51 (2), p.246-252 |
| issn | 0148-639X 1097-4598 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_1753498540 |
| source | MEDLINE; Wiley Online Library Journals Frontfile Complete |
| subjects | Action Potentials - physiology Animals Axons - pathology bands of Fontana Charcot-Marie-Tooth disease Charcot-Marie-Tooth Disease - genetics Charcot-Marie-Tooth Disease - metabolism Charcot-Marie-Tooth Disease - pathology Disease Models, Animal Electrophysiology Male Medical research Mice Mice, Inbred C57BL Mice, Neurologic Mutants Microscopy, Confocal Mutation - genetics Myelin Proteins - genetics Myelin Sheath - metabolism Myelin Sheath - pathology nerve stretch Neural Conduction - genetics Neural Conduction - physiology rat Rodents sciatic nerve Sciatic Nerve - metabolism Sciatic Nerve - physiopathology Stress, Mechanical |
| title | Normal nerve striations are altered in the trembler-J mouse, a model of charcot-marie-tooth disease |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-08T21%3A20%3A50IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Normal%20nerve%20striations%20are%20altered%20in%20the%20trembler-J%20mouse,%20a%20model%20of%20charcot-marie-tooth%20disease&rft.jtitle=Muscle%20&%20nerve&rft.au=Power,%20Bridget%20J.&rft.date=2015-02&rft.volume=51&rft.issue=2&rft.spage=246&rft.epage=252&rft.pages=246-252&rft.issn=0148-639X&rft.eissn=1097-4598&rft.coden=MUNEDE&rft_id=info:doi/10.1002/mus.24303&rft_dat=%3Cproquest_cross%3E1753498540%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=1644140974&rft_id=info:pmid/24890015&rfr_iscdi=true |