Connexin32 can restore hearing in connexin26 deficient mice
Functional gap junction channels composed of certain connexin proteins are essential for the function of the cochlea. Homozygous deficiency in the Gjb2 (mice) or GJB2 (human) gene coding for connexin26 (Cx26) in the cochlea leads to hearing impairment in mice and humans, respectively. Here we have s...
Saved in:
Published in | European journal of cell biology Vol. 90; no. 10; pp. 817 - 824 |
---|---|
Main Authors | , , , , , , |
Format | Journal Article |
Language | English |
Published |
Germany
Elsevier GmbH
01.10.2011
|
Subjects | |
Online Access | Get full text |
Cover
Loading…
Abstract | Functional gap junction channels composed of certain connexin proteins are essential for the function of the cochlea. Homozygous deficiency in the
Gjb2 (mice) or
GJB2 (human) gene coding for connexin26 (Cx26) in the cochlea leads to hearing impairment in mice and humans, respectively. Here we have studied the functional equivalence of Cx26 and connexin32 (Cx32) isoforms in the cochlea. We analyzed a conditional mouse mutant in which the
Gjb2 coding DNA was exchanged by LacZ DNA coding for the reporter protein beta-galactosidase. This allowed us to follow the unrestricted and cell type specific expression of
Gjb2 promoter activity. After inner ear specific, Otogelin-Cre recombinase mediated deletion of the loxP-site-flanked LacZ coding DNA, transcription of the
Gjb1 gene, coding for Cx32 was activated by the
Gjb2 promoter. Interbreeding of these mice with conditional
Gjb2 null mice resulted in animals in which Cx32 instead of Cx26 protein is expressed in the non-sensory epithelial network of the cochlea. When we analyzed the auditory function in these mice, we found that the expression of Cx32 protein is sufficient to support hearing in the absence of Cx26. Thus Cx32 can functionally replace Cx26 in the mouse cochlea resulting in almost normal hearing. |
---|---|
AbstractList | Functional gap junction channels composed of certain connexin proteins are essential for the function of the cochlea. Homozygous deficiency in the
Gjb2 (mice) or
GJB2 (human) gene coding for connexin26 (Cx26) in the cochlea leads to hearing impairment in mice and humans, respectively. Here we have studied the functional equivalence of Cx26 and connexin32 (Cx32) isoforms in the cochlea. We analyzed a conditional mouse mutant in which the
Gjb2 coding DNA was exchanged by LacZ DNA coding for the reporter protein beta-galactosidase. This allowed us to follow the unrestricted and cell type specific expression of
Gjb2 promoter activity. After inner ear specific, Otogelin-Cre recombinase mediated deletion of the loxP-site-flanked LacZ coding DNA, transcription of the
Gjb1 gene, coding for Cx32 was activated by the
Gjb2 promoter. Interbreeding of these mice with conditional
Gjb2 null mice resulted in animals in which Cx32 instead of Cx26 protein is expressed in the non-sensory epithelial network of the cochlea. When we analyzed the auditory function in these mice, we found that the expression of Cx32 protein is sufficient to support hearing in the absence of Cx26. Thus Cx32 can functionally replace Cx26 in the mouse cochlea resulting in almost normal hearing. Functional gap junction channels composed of certain connexin proteins are essential for the function of the cochlea. Homozygous deficiency in the Gjb2 (mice) or GJB2 (human) gene coding for connexin26 (Cx26) in the cochlea leads to hearing impairment in mice and humans, respectively. Here we have studied the functional equivalence of Cx26 and connexin32 (Cx32) isoforms in the cochlea. We analyzed a conditional mouse mutant in which the Gjb2 coding DNA was exchanged by LacZ DNA coding for the reporter protein beta-galactosidase. This allowed us to follow the unrestricted and cell type specific expression of Gjb2 promoter activity. After inner ear specific, Otogelin-Cre recombinase mediated deletion of the loxP-site-flanked LacZ coding DNA, transcription of the Gjb1 gene, coding for Cx32 was activated by the Gjb2 promoter. Interbreeding of these mice with conditional Gjb2 null mice resulted in animals in which Cx32 instead of Cx26 protein is expressed in the non-sensory epithelial network of the cochlea. When we analyzed the auditory function in these mice, we found that the expression of Cx32 protein is sufficient to support hearing in the absence of Cx26. Thus Cx32 can functionally replace Cx26 in the mouse cochlea resulting in almost normal hearing. |
Author | Degen, Joachim Willecke, Klaus Strenzke, Nicola Dicke, Nikolai Schütz, Melanie Jokwitz, Melanie Moser, Tobias |
Author_xml | – sequence: 1 givenname: Joachim surname: Degen fullname: Degen, Joachim organization: Institute of Genetics, University of Bonn, 53117 Bonn, Germany – sequence: 2 givenname: Melanie surname: Schütz fullname: Schütz, Melanie organization: Institute of Genetics, University of Bonn, 53117 Bonn, Germany – sequence: 3 givenname: Nikolai surname: Dicke fullname: Dicke, Nikolai organization: Institute of Genetics, University of Bonn, 53117 Bonn, Germany – sequence: 4 givenname: Nicola surname: Strenzke fullname: Strenzke, Nicola organization: University of Göttingen School of Medicine, Auditory Systems Physiology Group, Department of Otolaryngology, Göttingen, Germany – sequence: 5 givenname: Melanie surname: Jokwitz fullname: Jokwitz, Melanie organization: Institute of Genetics, University of Bonn, 53117 Bonn, Germany – sequence: 6 givenname: Tobias surname: Moser fullname: Moser, Tobias organization: University of Göttingen School of Medicine, Department of Otolaryngology and Center for Molecular Physiology of the Brain, Bernstein Center for Computational Neuroscience, 37075 Göttingen, Germany – sequence: 7 givenname: Klaus surname: Willecke fullname: Willecke, Klaus email: k.willecke@uni-bonn.de organization: Institute of Genetics, University of Bonn, 53117 Bonn, Germany |
BackLink | https://www.ncbi.nlm.nih.gov/pubmed/21813206$$D View this record in MEDLINE/PubMed |
BookMark | eNp9kMtOwzAQRS1URB_wAyxQfiDB48SpLdigipdUiQ2sLXs8AUfUqZyC4O9J1MKS1WzOvZp75mwSu0iMnQMvgEN92RbUoisEByi4LDiHIzaDGlQOQqsJm3FYQq7LUk7ZvO_bAZBK6xM2FaCgFLyesatVFyN9hViKDG3MEvW7LlH2RjaF-JqFmOGBEHXmqQkYKO6yTUA6ZceNfe_p7HAX7OXu9nn1kK-f7h9XN-scS6V3eSUbBeSWvlIIXrmqsrYhIt84qwF0XWmU3kniwnkErJ2Vy6pCxFLWHJpywcS-F1PX94kas01hY9O3AW5GE6Y1owkzmjBcmmHoELrYh7YfbkP-L_I7fQCu9wANr38GSqYfpyH5kAh3xnfhv_4fuDpxEA |
CitedBy_id | crossref_primary_10_3748_wjg_v23_i29_5345 crossref_primary_10_1016_j_bbamem_2011_10_016 crossref_primary_10_1016_j_bbamem_2012_06_024 crossref_primary_10_1242_jcs_214635 crossref_primary_10_3109_00016489_2015_1034880 crossref_primary_10_3389_fncel_2014_00354 crossref_primary_10_1016_j_jgg_2015_06_002 crossref_primary_10_3389_fncel_2015_00202 crossref_primary_10_1007_s00441_014_2029_z crossref_primary_10_1016_j_bbrc_2013_05_118 crossref_primary_10_1016_j_joto_2016_01_003 crossref_primary_10_3390_biomedicines10030589 crossref_primary_10_1093_hmg_ddv109 crossref_primary_10_1016_j_heares_2014_04_010 crossref_primary_10_1007_s00441_014_2024_4 crossref_primary_10_1590_1678_4685_gmb_2023_0170 |
Cites_doi | 10.1002/hep.510280622 10.1097/MOO.0b013e32830e20b0 10.1073/pnas.0501859102 10.1002/gene.20011 10.1038/ncb1205 10.1083/jcb.140.6.1453 10.1093/hmg/ddg116 10.1073/pnas.94.26.14450 10.1016/S0960-9822(02)00904-1 10.1002/cne.10916 10.1016/S0925-4773(02)00299-X 10.1073/pnas.93.18.9565 10.1073/pnas.0606855104 10.1523/JNEUROSCI.1577-09.2009 10.1093/hmg/ddg001 10.1042/BJ20051922 10.1002/cne.22117 10.1242/dev.072975 10.1074/jbc.M511235200 10.1093/hmg/ddq429 10.1016/S0006-291X(03)01166-5 10.1073/pnas.0800831105 10.1007/s007950070001 10.1016/j.bbrc.2009.05.023 10.1089/ars.2008.2128 10.1016/j.neuroscience.2008.08.027 |
ContentType | Journal Article |
Copyright | 2011 Elsevier GmbH Copyright © 2011 Elsevier GmbH. All rights reserved. |
Copyright_xml | – notice: 2011 Elsevier GmbH – notice: Copyright © 2011 Elsevier GmbH. All rights reserved. |
DBID | CGR CUY CVF ECM EIF NPM AAYXX CITATION |
DOI | 10.1016/j.ejcb.2011.05.001 |
DatabaseName | Medline MEDLINE MEDLINE (Ovid) MEDLINE MEDLINE PubMed CrossRef |
DatabaseTitle | MEDLINE Medline Complete MEDLINE with Full Text PubMed MEDLINE (Ovid) CrossRef |
DatabaseTitleList | MEDLINE |
Database_xml | – sequence: 1 dbid: NPM name: PubMed url: https://proxy.k.utb.cz/login?url=http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed sourceTypes: Index Database – sequence: 2 dbid: EIF name: MEDLINE url: https://proxy.k.utb.cz/login?url=https://www.webofscience.com/wos/medline/basic-search sourceTypes: Index Database |
DeliveryMethod | fulltext_linktorsrc |
Discipline | Biology |
EISSN | 1618-1298 |
EndPage | 824 |
ExternalDocumentID | 10_1016_j_ejcb_2011_05_001 21813206 S0171933511000987 |
Genre | Research Support, Non-U.S. Gov't Journal Article |
GroupedDBID | --- --K --M -~X .55 .~1 0R~ 0SF 1B1 1RT 1~. 1~5 29G 3O- 3V. 4.4 457 4CK 4G. 53G 5GY 5RE 5VS 7-5 71M 7X7 88A 88E 88I 8AF 8FE 8FH 8FI 8FJ 8P~ 8R4 8R5 AABNK AABVA AACTN AADPK AAEDT AAEDW AAFWJ AAIAV AAIKJ AAKOC AALCJ AALRI AAOAW AAQFI AAQXK AATLK AAXLA AAXUO ABCQJ ABFNM ABFRF ABGRD ABGSF ABJNI ABLJU ABMAC ABUDA ABUWG ABXDB ABYKQ ACDAQ ACGFO ACGOD ACPRK ACRLP ADBBV ADEZE ADKUU ADMUD ADQTV ADUVX AEBSH AEFWE AEHWI AEKER AENEX AEQOU AFFNX AFKRA AFKWA AFPKN AFTJW AFXIZ AGHFR AGRDE AGUBO AGWIK AGYEJ AHMBA AHPSJ AI. AIEXJ AIKHN AITUG AJBFU AJOXV ALMA_UNASSIGNED_HOLDINGS AMFUW AMRAJ ASPBG AVWKF AXJTR AZFZN AZQEC BBNVY BENPR BES BHPHI BKOJK BLXMC BPHCQ BVXVI CAG CBWCG CCPQU COF CS3 DOVZS DU5 DWQXO EBS EFJIC EFLBG EJD EO8 EO9 EP2 EP3 F5P FDB FEDTE FGOYB FIRID FNPLU FYGXN FYUFA G-Q GBLVA GNUQQ GROUPED_DOAJ HCIFZ HMCUK HVGLF HZ~ IH2 IHE J1W KOM LK8 M0L M1P M2P M2Q M41 M7P MO0 MOBAO N9A O-L O9- OAUVE OK1 OZT P-8 P-9 PC. PQQKQ PROAC PSQYO Q2X Q38 R2- RIG ROL RPZ S0X SDF SDG SES SEW SPCBC SSA SSN SSU SSZ T5J T5K UKHRP UNMZH VH1 WH7 X7M XJT ZGI ZXP ~G- AAXKI ADVLN AFJKZ AKRWK ALIPV CGR CUY CVF ECM EIF NPM AAYXX ACRPL ADNMO CITATION |
ID | FETCH-LOGICAL-c389t-45f81eb7d48c1d8b44aafeeedfba9119649c5db5e02bdc1c6ba5744ccc35601f3 |
IEDL.DBID | .~1 |
ISSN | 0171-9335 |
IngestDate | Fri Dec 06 05:54:54 EST 2024 Sat Nov 02 12:29:13 EDT 2024 Fri Feb 23 02:30:36 EST 2024 |
IsPeerReviewed | true |
IsScholarly | true |
Issue | 10 |
Keywords | Deafness Cx32 Gap junctions Cochlea Cx26 Otogelin-Cre Inner ear |
Language | English |
License | Copyright © 2011 Elsevier GmbH. All rights reserved. |
LinkModel | DirectLink |
MergedId | FETCHMERGED-LOGICAL-c389t-45f81eb7d48c1d8b44aafeeedfba9119649c5db5e02bdc1c6ba5744ccc35601f3 |
PMID | 21813206 |
PageCount | 8 |
ParticipantIDs | crossref_primary_10_1016_j_ejcb_2011_05_001 pubmed_primary_21813206 elsevier_sciencedirect_doi_10_1016_j_ejcb_2011_05_001 |
PublicationCentury | 2000 |
PublicationDate | 2011-10-01 |
PublicationDateYYYYMMDD | 2011-10-01 |
PublicationDate_xml | – month: 10 year: 2011 text: 2011-10-01 day: 01 |
PublicationDecade | 2010 |
PublicationPlace | Germany |
PublicationPlace_xml | – name: Germany |
PublicationTitle | European journal of cell biology |
PublicationTitleAlternate | Eur J Cell Biol |
PublicationYear | 2011 |
Publisher | Elsevier GmbH |
Publisher_xml | – name: Elsevier GmbH |
References | Ahmad, Tang, Chang, Qu, Hibshman, Li, Söhl, Willecke, Chen, Lin (bib0010) 2007; 104 Zhang, Tang, Ahmad, Sipp, Chen, Lin (bib0140) 2005; 102 Schütz, Auth, Gehrt, Bosen, Körber, Strenzke, Moser, Willecke (bib0115) 2011; 20 Kudo, Kure, Ikeda, Xia, Katori, Suzuki, Kojima, Ichinohe, Suzuki, Aoki (bib0080) 2003; 12 Cohen-Salmon, El-Amraoui, Leibovici, Petit (bib0030) 1997; 94 Laird (bib0085) 2006; 394 López-Bigas, Arbones, Estivill, Simonneau (bib0090) 2002; 2 Sun, Tang, Chang, Wang, Kong, Lin (bib0125) 2009; 516 Hibino, Kurachi (bib0065) 2006; 21 Inoshita, Iizuda, Okamura, Minekawa, Kojima, Furukawa, Kusunoki, Ikeda (bib0070) 2008; 156 Stümpel, Ott, Willecke, Jungermann (bib0120) 1998; 28 Teubner, Michel, Pesch, Lautermann, Cohen-Salmon, Söhl, Jahnke, Winterhager, Herberhold, Hardelin (bib0130) 2003; 12 Kikuchi, Adams, Miyabe, So, Kobayashi (bib0075) 2000; 33 Nelles, Bützler, Jung, Temme, Gabriel, Dahl, Traub, Stümpel, Jungermann, Zielasek (bib0100) 1996; 93 Cohen-Salmon, del Castillo, Petit (bib0025) 2005 Cohen-Salmon, Ott, Michel, Hardelin, Perfettini, Eybalin, Wu, Marcus, Wangemann, Willecke (bib0035) 2002; 12 Eckardt, Theis, Döring, Speidel, Willecke, Ott (bib0045) 2004; 38 Bedner, Niessen, Odermatt, Kretz, Willecke, Harz (bib0015) 2006; 281 Harris, Locke (bib0060) 2009 Ortolano, Di Pasquale, Crispino, Anselmi, Mammano, Chiorini (bib0110) 2008; 105 Beltramello, Piazza, Bukauskas, Pozzan, Mammano (bib0020) 2005; 7 Nickel, Forge (bib0105) 2008; 16 Wang, Chang, Tang, Sun, Zhou, Li, Lin (bib0135) 2009; 385 Dobrowolski, Willecke (bib0040) 2009; 11 Neef, Gehrt, Bulankina, Meyer, Riedel, Gregg, Strenzke, Moser (bib0095) 2009; 29 Dicke, N., Pielensticker, N., Degen, J., Tress, O., Hecker, J., Bald, T., Gellhaus, A., Winterhager, E., Willecke, K., 2011. Peripheral lymphangiogenesis in mice depends on ectodermal Connexin26 (Gjb2). J. Cell. Sci. in press. Gabriel, Jung, Bützler, Temme, Traub, Winterhager, Willecke (bib0055) 1998; 140 Forge, Becker, Casalotti, Edwards, Marziano, Nevill (bib0050) 2003; 467 Ahmad, Chen, Sun, Lin (bib0005) 2003; 307 Hibino (10.1016/j.ejcb.2011.05.001_bib0065) 2006; 21 10.1016/j.ejcb.2011.05.001_bib0145 Eckardt (10.1016/j.ejcb.2011.05.001_bib0045) 2004; 38 Kikuchi (10.1016/j.ejcb.2011.05.001_bib0075) 2000; 33 Ahmad (10.1016/j.ejcb.2011.05.001_bib0010) 2007; 104 Inoshita (10.1016/j.ejcb.2011.05.001_bib0070) 2008; 156 Ahmad (10.1016/j.ejcb.2011.05.001_bib0005) 2003; 307 Harris (10.1016/j.ejcb.2011.05.001_bib0060) 2009 Teubner (10.1016/j.ejcb.2011.05.001_bib0130) 2003; 12 Cohen-Salmon (10.1016/j.ejcb.2011.05.001_bib0035) 2002; 12 Wang (10.1016/j.ejcb.2011.05.001_bib0135) 2009; 385 Nickel (10.1016/j.ejcb.2011.05.001_bib0105) 2008; 16 Bedner (10.1016/j.ejcb.2011.05.001_bib0015) 2006; 281 Neef (10.1016/j.ejcb.2011.05.001_bib0095) 2009; 29 Forge (10.1016/j.ejcb.2011.05.001_bib0050) 2003; 467 Gabriel (10.1016/j.ejcb.2011.05.001_bib0055) 1998; 140 Schütz (10.1016/j.ejcb.2011.05.001_bib0115) 2011; 20 Zhang (10.1016/j.ejcb.2011.05.001_bib0140) 2005; 102 Sun (10.1016/j.ejcb.2011.05.001_bib0125) 2009; 516 Stümpel (10.1016/j.ejcb.2011.05.001_bib0120) 1998; 28 Nelles (10.1016/j.ejcb.2011.05.001_bib0100) 1996; 93 Beltramello (10.1016/j.ejcb.2011.05.001_bib0020) 2005; 7 Dobrowolski (10.1016/j.ejcb.2011.05.001_bib0040) 2009; 11 Kudo (10.1016/j.ejcb.2011.05.001_bib0080) 2003; 12 Cohen-Salmon (10.1016/j.ejcb.2011.05.001_bib0030) 1997; 94 López-Bigas (10.1016/j.ejcb.2011.05.001_bib0090) 2002; 2 Ortolano (10.1016/j.ejcb.2011.05.001_bib0110) 2008; 105 Cohen-Salmon (10.1016/j.ejcb.2011.05.001_bib0025) 2005 Laird (10.1016/j.ejcb.2011.05.001_bib0085) 2006; 394 |
References_xml | – volume: 21 start-page: 336 year: 2006 end-page: 345 ident: bib0065 article-title: Molecular and physiological bases of the K+ circulation in the mammalian inner ear publication-title: Physiology (Bethesda) contributor: fullname: Kurachi – volume: 12 start-page: 1106 year: 2002 end-page: 1111 ident: bib0035 article-title: Targeted ablation of connexin26 in the inner ear epithelial gap junction network causes hearing impairment and cell death publication-title: Curr. Biol. contributor: fullname: Willecke – volume: 394 start-page: 527 year: 2006 end-page: 543 ident: bib0085 article-title: Life cycle of connexins in health and disease publication-title: Biochem. J. contributor: fullname: Laird – volume: 12 start-page: 13 year: 2003 end-page: 21 ident: bib0130 article-title: Connexin30 (Gjb6)-deficiency causes severe hearing impairment and lack of endocochlear potential publication-title: Hum. Mol. Genet. contributor: fullname: Hardelin – volume: 385 start-page: 33 year: 2009 end-page: 37 ident: bib0135 article-title: Targeted connexin26 ablation arrests postnatal development of the organ of Corti publication-title: Biochem. Biophys. Res. Commun. contributor: fullname: Lin – volume: 93 start-page: 9565 year: 1996 end-page: 9570 ident: bib0100 article-title: Defective propagation of signals generated by sympathetic nerve stimulation in the liver of connexin32-deficient mice publication-title: Proc. Natl. Acad. Sci. U.S.A. contributor: fullname: Zielasek – volume: 7 start-page: 63 year: 2005 end-page: 69 ident: bib0020 article-title: Impaired permeability to Ins(1,4,5)P3 in a mutant connexin underlies recessive hereditary deafness publication-title: Nat. Cell. Biol. contributor: fullname: Mammano – year: 2009 ident: bib0060 article-title: Permeability of connexin channels publication-title: Connexins contributor: fullname: Locke – volume: 104 start-page: 1337 year: 2007 end-page: 1341 ident: bib0010 article-title: Restoration of connexin26 protein level in the cochlea completely rescues hearing in a mouse model of human connexin30-linked deafness publication-title: Proc. Natl. Acad. Sci. US.A. contributor: fullname: Lin – volume: 20 start-page: 28 year: 2011 end-page: 39 ident: bib0115 article-title: The connexin26 S17F mouse mutant represents a model for the human hereditary keratitis-ichthyosis-deafness syndrome publication-title: Hum. Mol. Genet. contributor: fullname: Willecke – start-page: 111 year: 2005 end-page: 134 ident: bib0025 article-title: Connexins responsible for hereditary deafness - the tale unfolds publication-title: Gap Junctions in Development and Disease contributor: fullname: Petit – volume: 12 start-page: 995 year: 2003 end-page: 1004 ident: bib0080 article-title: Transgenic expression of a dominant-negative connexin26 causes degeneration of the organ of Corti and non-syndromic deafness publication-title: Hum. Mol. Genet. contributor: fullname: Aoki – volume: 102 start-page: 15201 year: 2005 end-page: 15206 ident: bib0140 article-title: Gap junction-mediated intercellular biochemical coupling in cochlear supporting cells is required for normal cochlear functions publication-title: Proc. Natl. Acad. Sci. U.S.A. contributor: fullname: Lin – volume: 33 start-page: 51 year: 2000 end-page: 56 ident: bib0075 article-title: Potassium ion recycling pathway via gap junction systems in the mammalian cochlea and its interruption in hereditary nonsyndromic deafness publication-title: Med. Electron Microsc. contributor: fullname: Kobayashi – volume: 94 start-page: 14450 year: 1997 end-page: 14455 ident: bib0030 article-title: Otogelin: a glycoprotein specific to the acellular membranes of the inner ear publication-title: Proc. Natl. Acad. Sci. U.S.A. contributor: fullname: Petit – volume: 11 start-page: 283 year: 2009 end-page: 295 ident: bib0040 article-title: Connexin-caused genetic diseases and corresponding mouse models publication-title: Antioxid. Redox Signal. contributor: fullname: Willecke – volume: 307 start-page: 362 year: 2003 end-page: 368 ident: bib0005 article-title: Connexins 26 and 30 are co-assembled to form gap junctions in the cochlea of mice publication-title: Biochem. Biophys. Res. Commun. contributor: fullname: Lin – volume: 38 start-page: 159 year: 2004 end-page: 165 ident: bib0045 article-title: Spontaneous ectopic recombination in cell-type-specific Cre mice removes loxP-flanked marker cassettes in vivo publication-title: Genesis contributor: fullname: Ott – volume: 281 start-page: 6673 year: 2006 end-page: 6681 ident: bib0015 article-title: Selective permeability of different connexin channels to the second messenger cyclic AMP publication-title: J. Biol. Chem. contributor: fullname: Harz – volume: 16 start-page: 452 year: 2008 end-page: 457 ident: bib0105 article-title: Gap junctions and connexins in the inner ear: their roles in homeostasis and deafness publication-title: Curr. Opin. Otolaryngol. Head Neck Surg. contributor: fullname: Forge – volume: 140 start-page: 1453 year: 1998 end-page: 1461 ident: bib0055 article-title: Transplacental uptake of glucose is decreased in embryonic lethal connexin26-deficient mice publication-title: J. Cell. Biol. contributor: fullname: Willecke – volume: 516 start-page: 569 year: 2009 end-page: 579 ident: bib0125 article-title: Connexin30 null and conditional connexin26 null mice display distinct pattern and time course of cellular degeneration in the cochlea publication-title: J. Comp. Neurol. contributor: fullname: Lin – volume: 2 start-page: 113 year: 2002 end-page: 117 ident: bib0090 article-title: Expression profiles of the connexin genes Gjb1 and Gjb3, in the developing mouse cochlea publication-title: Gene Expr. Patterns contributor: fullname: Simonneau – volume: 467 start-page: 207 year: 2003 end-page: 231 ident: bib0050 article-title: Gap junctions in the inner ear: comparison of distribution patterns in different vertebrates and assessement of connexin composition in mammals publication-title: J. Comp. Neurol. contributor: fullname: Nevill – volume: 28 start-page: 1616 year: 1998 end-page: 1620 ident: bib0120 article-title: Connexin 32 gap junctions enhance stimulation of glucose output by glucagon and noradrenaline in mouse liver publication-title: Hepatology contributor: fullname: Jungermann – volume: 156 start-page: 1039 year: 2008 end-page: 1047 ident: bib0070 article-title: Postnatal development of the organ of Corti in dominant-negative publication-title: Neuroscience contributor: fullname: Ikeda – volume: 29 start-page: 10730 year: 2009 end-page: 10740 ident: bib0095 article-title: The Ca2+ channel subunit beta2 regulates Ca2+ channel abundance and function in inner hair cells and is required for hearing publication-title: J. Neurosci. contributor: fullname: Moser – volume: 105 start-page: 18776 year: 2008 end-page: 18781 ident: bib0110 article-title: Coordinated control of connexin 26 and connexin 30 at the regulatory and functional level in the inner ear publication-title: Proc. Natl. Acad. Sci. U.S.A. contributor: fullname: Chiorini – volume: 28 start-page: 1616 year: 1998 ident: 10.1016/j.ejcb.2011.05.001_bib0120 article-title: Connexin 32 gap junctions enhance stimulation of glucose output by glucagon and noradrenaline in mouse liver publication-title: Hepatology doi: 10.1002/hep.510280622 contributor: fullname: Stümpel – volume: 21 start-page: 336 year: 2006 ident: 10.1016/j.ejcb.2011.05.001_bib0065 article-title: Molecular and physiological bases of the K+ circulation in the mammalian inner ear publication-title: Physiology (Bethesda) contributor: fullname: Hibino – volume: 16 start-page: 452 year: 2008 ident: 10.1016/j.ejcb.2011.05.001_bib0105 article-title: Gap junctions and connexins in the inner ear: their roles in homeostasis and deafness publication-title: Curr. Opin. Otolaryngol. Head Neck Surg. doi: 10.1097/MOO.0b013e32830e20b0 contributor: fullname: Nickel – volume: 102 start-page: 15201 year: 2005 ident: 10.1016/j.ejcb.2011.05.001_bib0140 article-title: Gap junction-mediated intercellular biochemical coupling in cochlear supporting cells is required for normal cochlear functions publication-title: Proc. Natl. Acad. Sci. U.S.A. doi: 10.1073/pnas.0501859102 contributor: fullname: Zhang – volume: 38 start-page: 159 year: 2004 ident: 10.1016/j.ejcb.2011.05.001_bib0045 article-title: Spontaneous ectopic recombination in cell-type-specific Cre mice removes loxP-flanked marker cassettes in vivo publication-title: Genesis doi: 10.1002/gene.20011 contributor: fullname: Eckardt – volume: 7 start-page: 63 year: 2005 ident: 10.1016/j.ejcb.2011.05.001_bib0020 article-title: Impaired permeability to Ins(1,4,5)P3 in a mutant connexin underlies recessive hereditary deafness publication-title: Nat. Cell. Biol. doi: 10.1038/ncb1205 contributor: fullname: Beltramello – volume: 140 start-page: 1453 year: 1998 ident: 10.1016/j.ejcb.2011.05.001_bib0055 article-title: Transplacental uptake of glucose is decreased in embryonic lethal connexin26-deficient mice publication-title: J. Cell. Biol. doi: 10.1083/jcb.140.6.1453 contributor: fullname: Gabriel – volume: 12 start-page: 995 year: 2003 ident: 10.1016/j.ejcb.2011.05.001_bib0080 article-title: Transgenic expression of a dominant-negative connexin26 causes degeneration of the organ of Corti and non-syndromic deafness publication-title: Hum. Mol. Genet. doi: 10.1093/hmg/ddg116 contributor: fullname: Kudo – volume: 94 start-page: 14450 year: 1997 ident: 10.1016/j.ejcb.2011.05.001_bib0030 article-title: Otogelin: a glycoprotein specific to the acellular membranes of the inner ear publication-title: Proc. Natl. Acad. Sci. U.S.A. doi: 10.1073/pnas.94.26.14450 contributor: fullname: Cohen-Salmon – volume: 12 start-page: 1106 year: 2002 ident: 10.1016/j.ejcb.2011.05.001_bib0035 article-title: Targeted ablation of connexin26 in the inner ear epithelial gap junction network causes hearing impairment and cell death publication-title: Curr. Biol. doi: 10.1016/S0960-9822(02)00904-1 contributor: fullname: Cohen-Salmon – volume: 467 start-page: 207 year: 2003 ident: 10.1016/j.ejcb.2011.05.001_bib0050 article-title: Gap junctions in the inner ear: comparison of distribution patterns in different vertebrates and assessement of connexin composition in mammals publication-title: J. Comp. Neurol. doi: 10.1002/cne.10916 contributor: fullname: Forge – volume: 2 start-page: 113 year: 2002 ident: 10.1016/j.ejcb.2011.05.001_bib0090 article-title: Expression profiles of the connexin genes Gjb1 and Gjb3, in the developing mouse cochlea publication-title: Gene Expr. Patterns doi: 10.1016/S0925-4773(02)00299-X contributor: fullname: López-Bigas – volume: 93 start-page: 9565 year: 1996 ident: 10.1016/j.ejcb.2011.05.001_bib0100 article-title: Defective propagation of signals generated by sympathetic nerve stimulation in the liver of connexin32-deficient mice publication-title: Proc. Natl. Acad. Sci. U.S.A. doi: 10.1073/pnas.93.18.9565 contributor: fullname: Nelles – year: 2009 ident: 10.1016/j.ejcb.2011.05.001_bib0060 article-title: Permeability of connexin channels contributor: fullname: Harris – volume: 104 start-page: 1337 year: 2007 ident: 10.1016/j.ejcb.2011.05.001_bib0010 article-title: Restoration of connexin26 protein level in the cochlea completely rescues hearing in a mouse model of human connexin30-linked deafness publication-title: Proc. Natl. Acad. Sci. US.A. doi: 10.1073/pnas.0606855104 contributor: fullname: Ahmad – volume: 29 start-page: 10730 year: 2009 ident: 10.1016/j.ejcb.2011.05.001_bib0095 article-title: The Ca2+ channel subunit beta2 regulates Ca2+ channel abundance and function in inner hair cells and is required for hearing publication-title: J. Neurosci. doi: 10.1523/JNEUROSCI.1577-09.2009 contributor: fullname: Neef – volume: 12 start-page: 13 year: 2003 ident: 10.1016/j.ejcb.2011.05.001_bib0130 article-title: Connexin30 (Gjb6)-deficiency causes severe hearing impairment and lack of endocochlear potential publication-title: Hum. Mol. Genet. doi: 10.1093/hmg/ddg001 contributor: fullname: Teubner – volume: 394 start-page: 527 year: 2006 ident: 10.1016/j.ejcb.2011.05.001_bib0085 article-title: Life cycle of connexins in health and disease publication-title: Biochem. J. doi: 10.1042/BJ20051922 contributor: fullname: Laird – volume: 516 start-page: 569 year: 2009 ident: 10.1016/j.ejcb.2011.05.001_bib0125 article-title: Connexin30 null and conditional connexin26 null mice display distinct pattern and time course of cellular degeneration in the cochlea publication-title: J. Comp. Neurol. doi: 10.1002/cne.22117 contributor: fullname: Sun – ident: 10.1016/j.ejcb.2011.05.001_bib0145 doi: 10.1242/dev.072975 – volume: 281 start-page: 6673 year: 2006 ident: 10.1016/j.ejcb.2011.05.001_bib0015 article-title: Selective permeability of different connexin channels to the second messenger cyclic AMP publication-title: J. Biol. Chem. doi: 10.1074/jbc.M511235200 contributor: fullname: Bedner – volume: 20 start-page: 28 year: 2011 ident: 10.1016/j.ejcb.2011.05.001_bib0115 article-title: The connexin26 S17F mouse mutant represents a model for the human hereditary keratitis-ichthyosis-deafness syndrome publication-title: Hum. Mol. Genet. doi: 10.1093/hmg/ddq429 contributor: fullname: Schütz – volume: 307 start-page: 362 year: 2003 ident: 10.1016/j.ejcb.2011.05.001_bib0005 article-title: Connexins 26 and 30 are co-assembled to form gap junctions in the cochlea of mice publication-title: Biochem. Biophys. Res. Commun. doi: 10.1016/S0006-291X(03)01166-5 contributor: fullname: Ahmad – volume: 105 start-page: 18776 year: 2008 ident: 10.1016/j.ejcb.2011.05.001_bib0110 article-title: Coordinated control of connexin 26 and connexin 30 at the regulatory and functional level in the inner ear publication-title: Proc. Natl. Acad. Sci. U.S.A. doi: 10.1073/pnas.0800831105 contributor: fullname: Ortolano – volume: 33 start-page: 51 year: 2000 ident: 10.1016/j.ejcb.2011.05.001_bib0075 article-title: Potassium ion recycling pathway via gap junction systems in the mammalian cochlea and its interruption in hereditary nonsyndromic deafness publication-title: Med. Electron Microsc. doi: 10.1007/s007950070001 contributor: fullname: Kikuchi – volume: 385 start-page: 33 year: 2009 ident: 10.1016/j.ejcb.2011.05.001_bib0135 article-title: Targeted connexin26 ablation arrests postnatal development of the organ of Corti publication-title: Biochem. Biophys. Res. Commun. doi: 10.1016/j.bbrc.2009.05.023 contributor: fullname: Wang – volume: 11 start-page: 283 year: 2009 ident: 10.1016/j.ejcb.2011.05.001_bib0040 article-title: Connexin-caused genetic diseases and corresponding mouse models publication-title: Antioxid. Redox Signal. doi: 10.1089/ars.2008.2128 contributor: fullname: Dobrowolski – start-page: 111 year: 2005 ident: 10.1016/j.ejcb.2011.05.001_bib0025 article-title: Connexins responsible for hereditary deafness - the tale unfolds contributor: fullname: Cohen-Salmon – volume: 156 start-page: 1039 year: 2008 ident: 10.1016/j.ejcb.2011.05.001_bib0070 article-title: Postnatal development of the organ of Corti in dominant-negative GJB2 transgenic mice publication-title: Neuroscience doi: 10.1016/j.neuroscience.2008.08.027 contributor: fullname: Inoshita |
SSID | ssj0015899 |
Score | 2.1012087 |
Snippet | Functional gap junction channels composed of certain connexin proteins are essential for the function of the cochlea. Homozygous deficiency in the
Gjb2 (mice)... Functional gap junction channels composed of certain connexin proteins are essential for the function of the cochlea. Homozygous deficiency in the Gjb2 (mice)... |
SourceID | crossref pubmed elsevier |
SourceType | Aggregation Database Index Database Publisher |
StartPage | 817 |
SubjectTerms | Animals beta-Galactosidase - biosynthesis beta-Galactosidase - genetics Cochlea Connexin 26 Connexin 30 Connexins - deficiency Connexins - genetics Connexins - metabolism Cx26 Cx32 Deafness Deafness - genetics Evoked Potentials, Auditory, Brain Stem Gap Junction beta-1 Protein Gap junctions Gene Dosage Gene Expression Genes, Reporter Genetic Engineering Inner ear Membrane Glycoproteins - genetics Membrane Glycoproteins - metabolism Mice Mice, Knockout Mice, Transgenic Otogelin-Cre Protein Isoforms - genetics Protein Isoforms - metabolism Recombinant Proteins - genetics Recombinant Proteins - metabolism Spiral Ligament of Cochlea - metabolism Stria Vascularis - cytology Stria Vascularis - metabolism |
Title | Connexin32 can restore hearing in connexin26 deficient mice |
URI | https://dx.doi.org/10.1016/j.ejcb.2011.05.001 https://www.ncbi.nlm.nih.gov/pubmed/21813206 |
Volume | 90 |
hasFullText | 1 |
inHoldings | 1 |
isFullTextHit | |
isPrint | |
link | http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwnV1LS8NAEB5KRfAivq2PsgdvEptNdtIsnkqxVMVetNBbyD4C7SGWUkEv_nZ3kk3Ri4LXMHnwTZjH8s18AFc5VzrNNZ24YxyIFEUg46QIVJjmKAouUNJw8tMkGU_FwwxnLRg2szBEq_Sxv47pVbT2V3oezd5yPu8906YX144jLT0LXetME-wYkorBzeeG5sExrTQkyTggaz84U3O87EIrv8aTjlb4H8npW-YZ7cGuLxnZoP6qfWjZ8gC2axHJj0O4rbgq7_MyjpjDia0qrRjLSKra5SU2L5n2FlHCjKWVES7TMNKhP4Lp6O5lOA68JEKgXWWxDgQWKbeqb0SquUmVEHleWJfnCpW7sCUTITUahTaMlNFcJyrHvhBa65haryI-hnb5WtpTYEq50saiNUKFwrlEOqclVhorI2u0jDtw3WCRLevNF1lDCVtkhFxGyGUhEi-uA9jAlf3wX-ZC86_3ndTYbt5BNUcchcnZP594DjtRw9PjF9Ber97spSsc1qpb_Rld2BrcP44nX8LjwGY |
link.rule.ids | 314,780,784,4502,24116,27924,27925,45585,45679 |
linkProvider | Elsevier |
linkToHtml | http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwnV1LS8NAEB5qRfQivq3PPXiT2Gyym2bxJGKp2vZiC70t2UegPcRSKujF3-5Osil6UfAaJpvwzTLzzfLtDMBVRpVOM40n7jwOWMpZIOIkD1SYZpzllHGBl5MHw6Q3Zk8TPmnAfX0XBmWVPvZXMb2M1v5J26PZnk-n7Rfs9OLKcY5Nz0JXOq_BOuOO_bpNffO50nlQnpZDJNE6QHN_c6YSedmZVr6PJ56t0D-y07fU092Bbc8ZyV31W7vQsMUebFRTJD_24bYUq7xPizgiDiiyKIfFWIKzql1iItOCaG8RJcRY7BnhUg3BQfQHMO4-jO57gZ-JEGhHLZYB43lKreoYlmpqUsVYluXWJbpcZS5uiYQJzY3iNoyU0VQnKuMdxrTWMdZeeXwIzeK1sMdAlHLcxnJrmAqZ84lwXkusMFZE1mgRt-C6xkLOq9YXstaEzSQiJxE5GXIUxrWA13DJHw6ULjb_-t5Rhe3qG0g64ihMTv654iVs9kaDvuw_Dp9PYSuqRXv0DJrLxZs9dyxiqS7KXfIFi5rCAw |
openUrl | ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Connexin32+can+restore+hearing+in+connexin26+deficient+mice&rft.jtitle=European+journal+of+cell+biology&rft.au=Degen%2C+Joachim&rft.au=Sch%C3%BCtz%2C+Melanie&rft.au=Dicke%2C+Nikolai&rft.au=Strenzke%2C+Nicola&rft.date=2011-10-01&rft.eissn=1618-1298&rft.volume=90&rft.issue=10&rft.spage=817&rft_id=info:doi/10.1016%2Fj.ejcb.2011.05.001&rft_id=info%3Apmid%2F21813206&rft.externalDocID=21813206 |
thumbnail_l | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/lc.gif&issn=0171-9335&client=summon |
thumbnail_m | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/mc.gif&issn=0171-9335&client=summon |
thumbnail_s | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/sc.gif&issn=0171-9335&client=summon |