Strain-dependent brain defects in mouse models of primary ciliary dyskinesia with mutations in Pcdp1 and Spef2
Highlights • Mice lacking Pcdp1 and Spef2 have hydrocephalus with damage to multiple cell types. • Morphological defects are more severe on the C57BL/6J background than 129S6/SvEvTac. • Mutants have a defect in cilia-driven cerebrospinal fluid flow on both strains. • Genetic modifiers likely influen...
Gespeichert in:
| Veröffentlicht in: | Neuroscience 2014-09, Vol.277, p.552-567 |
|---|---|
| Hauptverfasser: | , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 567 |
|---|---|
| container_issue | |
| container_start_page | 552 |
| container_title | Neuroscience |
| container_volume | 277 |
| creator | Finn, R Evans, C.C Lee, L |
| description | Highlights • Mice lacking Pcdp1 and Spef2 have hydrocephalus with damage to multiple cell types. • Morphological defects are more severe on the C57BL/6J background than 129S6/SvEvTac. • Mutants have a defect in cilia-driven cerebrospinal fluid flow on both strains. • Genetic modifiers likely influence the severity of hydrocephalus in these models. |
| doi_str_mv | 10.1016/j.neuroscience.2014.07.029 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_4164581</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><els_id>1_s2_0_S0306452214006022</els_id><sourcerecordid>1562430789</sourcerecordid><originalsourceid>FETCH-LOGICAL-c575t-ac880c0217094b08f5a7f336ddad6aee009797ee4f845e69e53c05218d045c253</originalsourceid><addsrcrecordid>eNqNkk1v1DAQhiMEokvhLyCLE5eE8XfCoRJqKSBVAmnhbHntCfU2cZY4Kdp_X4ddqsIFfPDY8juvx36mKF5RqChQ9WZbRZzHIbmA0WHFgIoKdAWseVSsaK15qaUQj4sVcFClkIydFM9S2kIeUvCnxQmToDkIviriehptiKXHHUaPcSKbZU88tuimRPKyH-aEefbYJTK0ZDeG3o574kIXluj36SZETMGSn2G6Jv082SkM8VfyF-d3lNjoyXqHLXtePGltl_DFMZ4W3y7ffz3_WF59_vDp_N1V6aSWU2ldXYMDRjU0YgN1K61uOVfeW68sIkCjG40o2lpIVA1K7kAyWnsQ0jHJT4uzg-9u3vToXX7YaDtzLN0MNpg_T2K4Nt-HWyOoErKm2eD10WAcfsyYJtOH5LDrbMT8H4Yq3jS1oiD_LZWKCQ66brL07UHqMr40YntfEQWzsDVb85CtWdga0CazzckvH77pPvU3zCy4OAgyKLwNOJqjjQ9jhmn8EP7vnrO_bFwXYnC2u8E9pu0wjzGzM9QkZsCsly5bmowKAAWM8TvYZNL-</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1562430789</pqid></control><display><type>article</type><title>Strain-dependent brain defects in mouse models of primary ciliary dyskinesia with mutations in Pcdp1 and Spef2</title><source>MEDLINE</source><source>Elsevier ScienceDirect Journals</source><creator>Finn, R ; Evans, C.C ; Lee, L</creator><creatorcontrib>Finn, R ; Evans, C.C ; Lee, L</creatorcontrib><description>Highlights • Mice lacking Pcdp1 and Spef2 have hydrocephalus with damage to multiple cell types. • Morphological defects are more severe on the C57BL/6J background than 129S6/SvEvTac. • Mutants have a defect in cilia-driven cerebrospinal fluid flow on both strains. • Genetic modifiers likely influence the severity of hydrocephalus in these models.</description><identifier>ISSN: 0306-4522</identifier><identifier>EISSN: 1873-7544</identifier><identifier>DOI: 10.1016/j.neuroscience.2014.07.029</identifier><identifier>PMID: 25073043</identifier><language>eng</language><publisher>United States: Elsevier Ltd</publisher><subject>Animals ; Brain - pathology ; Brain - physiopathology ; Cerebrospinal Fluid - physiology ; Cfap221 ; cilia ; Disease Models, Animal ; Genetic Predisposition to Disease ; hydrocephalus ; Hydrocephalus - pathology ; Hydrocephalus - physiopathology ; Immunohistochemistry ; Kartagener Syndrome - pathology ; Kartagener Syndrome - physiopathology ; Mice, 129 Strain ; Mice, Inbred C57BL ; Mice, Transgenic ; Mutation ; Neurology ; Neurons - pathology ; Neurons - physiology ; Oligodendroglia - pathology ; Oligodendroglia - physiology ; Pcdp1 ; primary ciliary dyskinesia ; Proteins - genetics ; Proteins - metabolism ; Severity of Illness Index ; Species Specificity ; Spef2</subject><ispartof>Neuroscience, 2014-09, Vol.277, p.552-567</ispartof><rights>IBRO</rights><rights>2014 IBRO</rights><rights>Copyright © 2014 IBRO. Published by Elsevier Ltd. All rights reserved.</rights><rights>2014 IBRO. Published by Elsevier Ltd. All rights reserved. 2014</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c575t-ac880c0217094b08f5a7f336ddad6aee009797ee4f845e69e53c05218d045c253</citedby><cites>FETCH-LOGICAL-c575t-ac880c0217094b08f5a7f336ddad6aee009797ee4f845e69e53c05218d045c253</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S0306452214006022$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>230,314,776,780,881,3537,27901,27902,65306</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/25073043$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Finn, R</creatorcontrib><creatorcontrib>Evans, C.C</creatorcontrib><creatorcontrib>Lee, L</creatorcontrib><title>Strain-dependent brain defects in mouse models of primary ciliary dyskinesia with mutations in Pcdp1 and Spef2</title><title>Neuroscience</title><addtitle>Neuroscience</addtitle><description>Highlights • Mice lacking Pcdp1 and Spef2 have hydrocephalus with damage to multiple cell types. • Morphological defects are more severe on the C57BL/6J background than 129S6/SvEvTac. • Mutants have a defect in cilia-driven cerebrospinal fluid flow on both strains. • Genetic modifiers likely influence the severity of hydrocephalus in these models.</description><subject>Animals</subject><subject>Brain - pathology</subject><subject>Brain - physiopathology</subject><subject>Cerebrospinal Fluid - physiology</subject><subject>Cfap221</subject><subject>cilia</subject><subject>Disease Models, Animal</subject><subject>Genetic Predisposition to Disease</subject><subject>hydrocephalus</subject><subject>Hydrocephalus - pathology</subject><subject>Hydrocephalus - physiopathology</subject><subject>Immunohistochemistry</subject><subject>Kartagener Syndrome - pathology</subject><subject>Kartagener Syndrome - physiopathology</subject><subject>Mice, 129 Strain</subject><subject>Mice, Inbred C57BL</subject><subject>Mice, Transgenic</subject><subject>Mutation</subject><subject>Neurology</subject><subject>Neurons - pathology</subject><subject>Neurons - physiology</subject><subject>Oligodendroglia - pathology</subject><subject>Oligodendroglia - physiology</subject><subject>Pcdp1</subject><subject>primary ciliary dyskinesia</subject><subject>Proteins - genetics</subject><subject>Proteins - metabolism</subject><subject>Severity of Illness Index</subject><subject>Species Specificity</subject><subject>Spef2</subject><issn>0306-4522</issn><issn>1873-7544</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkk1v1DAQhiMEokvhLyCLE5eE8XfCoRJqKSBVAmnhbHntCfU2cZY4Kdp_X4ddqsIFfPDY8juvx36mKF5RqChQ9WZbRZzHIbmA0WHFgIoKdAWseVSsaK15qaUQj4sVcFClkIydFM9S2kIeUvCnxQmToDkIviriehptiKXHHUaPcSKbZU88tuimRPKyH-aEefbYJTK0ZDeG3o574kIXluj36SZETMGSn2G6Jv082SkM8VfyF-d3lNjoyXqHLXtePGltl_DFMZ4W3y7ffz3_WF59_vDp_N1V6aSWU2ldXYMDRjU0YgN1K61uOVfeW68sIkCjG40o2lpIVA1K7kAyWnsQ0jHJT4uzg-9u3vToXX7YaDtzLN0MNpg_T2K4Nt-HWyOoErKm2eD10WAcfsyYJtOH5LDrbMT8H4Yq3jS1oiD_LZWKCQ66brL07UHqMr40YntfEQWzsDVb85CtWdga0CazzckvH77pPvU3zCy4OAgyKLwNOJqjjQ9jhmn8EP7vnrO_bFwXYnC2u8E9pu0wjzGzM9QkZsCsly5bmowKAAWM8TvYZNL-</recordid><startdate>20140926</startdate><enddate>20140926</enddate><creator>Finn, R</creator><creator>Evans, C.C</creator><creator>Lee, L</creator><general>Elsevier Ltd</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><scope>7TK</scope><scope>5PM</scope></search><sort><creationdate>20140926</creationdate><title>Strain-dependent brain defects in mouse models of primary ciliary dyskinesia with mutations in Pcdp1 and Spef2</title><author>Finn, R ; Evans, C.C ; Lee, L</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c575t-ac880c0217094b08f5a7f336ddad6aee009797ee4f845e69e53c05218d045c253</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2014</creationdate><topic>Animals</topic><topic>Brain - pathology</topic><topic>Brain - physiopathology</topic><topic>Cerebrospinal Fluid - physiology</topic><topic>Cfap221</topic><topic>cilia</topic><topic>Disease Models, Animal</topic><topic>Genetic Predisposition to Disease</topic><topic>hydrocephalus</topic><topic>Hydrocephalus - pathology</topic><topic>Hydrocephalus - physiopathology</topic><topic>Immunohistochemistry</topic><topic>Kartagener Syndrome - pathology</topic><topic>Kartagener Syndrome - physiopathology</topic><topic>Mice, 129 Strain</topic><topic>Mice, Inbred C57BL</topic><topic>Mice, Transgenic</topic><topic>Mutation</topic><topic>Neurology</topic><topic>Neurons - pathology</topic><topic>Neurons - physiology</topic><topic>Oligodendroglia - pathology</topic><topic>Oligodendroglia - physiology</topic><topic>Pcdp1</topic><topic>primary ciliary dyskinesia</topic><topic>Proteins - genetics</topic><topic>Proteins - metabolism</topic><topic>Severity of Illness Index</topic><topic>Species Specificity</topic><topic>Spef2</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Finn, R</creatorcontrib><creatorcontrib>Evans, C.C</creatorcontrib><creatorcontrib>Lee, L</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><collection>Neurosciences Abstracts</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Neuroscience</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Finn, R</au><au>Evans, C.C</au><au>Lee, L</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Strain-dependent brain defects in mouse models of primary ciliary dyskinesia with mutations in Pcdp1 and Spef2</atitle><jtitle>Neuroscience</jtitle><addtitle>Neuroscience</addtitle><date>2014-09-26</date><risdate>2014</risdate><volume>277</volume><spage>552</spage><epage>567</epage><pages>552-567</pages><issn>0306-4522</issn><eissn>1873-7544</eissn><abstract>Highlights • Mice lacking Pcdp1 and Spef2 have hydrocephalus with damage to multiple cell types. • Morphological defects are more severe on the C57BL/6J background than 129S6/SvEvTac. • Mutants have a defect in cilia-driven cerebrospinal fluid flow on both strains. • Genetic modifiers likely influence the severity of hydrocephalus in these models.</abstract><cop>United States</cop><pub>Elsevier Ltd</pub><pmid>25073043</pmid><doi>10.1016/j.neuroscience.2014.07.029</doi><tpages>16</tpages><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0306-4522 |
| ispartof | Neuroscience, 2014-09, Vol.277, p.552-567 |
| issn | 0306-4522 1873-7544 |
| language | eng |
| recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_4164581 |
| source | MEDLINE; Elsevier ScienceDirect Journals |
| subjects | Animals Brain - pathology Brain - physiopathology Cerebrospinal Fluid - physiology Cfap221 cilia Disease Models, Animal Genetic Predisposition to Disease hydrocephalus Hydrocephalus - pathology Hydrocephalus - physiopathology Immunohistochemistry Kartagener Syndrome - pathology Kartagener Syndrome - physiopathology Mice, 129 Strain Mice, Inbred C57BL Mice, Transgenic Mutation Neurology Neurons - pathology Neurons - physiology Oligodendroglia - pathology Oligodendroglia - physiology Pcdp1 primary ciliary dyskinesia Proteins - genetics Proteins - metabolism Severity of Illness Index Species Specificity Spef2 |
| title | Strain-dependent brain defects in mouse models of primary ciliary dyskinesia with mutations in Pcdp1 and Spef2 |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-03T04%3A43%3A31IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Strain-dependent%20brain%20defects%20in%20mouse%20models%20of%20primary%20ciliary%20dyskinesia%20with%20mutations%20in%20Pcdp1%20and%20Spef2&rft.jtitle=Neuroscience&rft.au=Finn,%20R&rft.date=2014-09-26&rft.volume=277&rft.spage=552&rft.epage=567&rft.pages=552-567&rft.issn=0306-4522&rft.eissn=1873-7544&rft_id=info:doi/10.1016/j.neuroscience.2014.07.029&rft_dat=%3Cproquest_pubme%3E1562430789%3C/proquest_pubme%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=1562430789&rft_id=info:pmid/25073043&rft_els_id=1_s2_0_S0306452214006022&rfr_iscdi=true |