PMID- 21813206
OWN - NLM
STAT- MEDLINE
DCOM- 20111227
LR  - 20231213
IS  - 1618-1298 (Electronic)
IS  - 0171-9335 (Linking)
VI  - 90
IP  - 10
DP  - 2011 Oct
TI  - Connexin32 can restore hearing in connexin26 deficient mice.
PG  - 817-24
LID - 10.1016/j.ejcb.2011.05.001 [doi]
AB  - 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.
CI  - Copyright (c) 2011 Elsevier GmbH. All rights reserved.
FAU - Degen, Joachim
AU  - Degen J
AD  - Institute of Genetics, University of Bonn, 53117 Bonn, Germany.
FAU - Schutz, Melanie
AU  - Schutz M
FAU - Dicke, Nikolai
AU  - Dicke N
FAU - Strenzke, Nicola
AU  - Strenzke N
FAU - Jokwitz, Melanie
AU  - Jokwitz M
FAU - Moser, Tobias
AU  - Moser T
FAU - Willecke, Klaus
AU  - Willecke K
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20110802
PL  - Germany
TA  - Eur J Cell Biol
JT  - European journal of cell biology
JID - 7906240
RN  - 0 (Connexin 30)
RN  - 0 (Connexins)
RN  - 0 (GJB2 protein, human)
RN  - 0 (Gjb2 protein, mouse)
RN  - 0 (Gjb6 protein, mouse)
RN  - 0 (Membrane Glycoproteins)
RN  - 0 (Otog protein, mouse)
RN  - 0 (Protein Isoforms)
RN  - 0 (Recombinant Proteins)
RN  - 127120-53-0 (Connexin 26)
RN  - EC 3.2.1.23 (beta-Galactosidase)
SB  - IM
MH  - Animals
MH  - Connexin 26
MH  - Connexin 30
MH  - Connexins/*deficiency/*genetics/metabolism
MH  - Deafness/*genetics
MH  - Evoked Potentials, Auditory, Brain Stem
MH  - Gene Dosage
MH  - Gene Expression
MH  - Genes, Reporter
MH  - Genetic Engineering
MH  - Membrane Glycoproteins/genetics/metabolism
MH  - Mice
MH  - Mice, Knockout
MH  - Mice, Transgenic
MH  - Protein Isoforms/genetics/metabolism
MH  - Recombinant Proteins/*genetics/metabolism
MH  - Spiral Ligament of Cochlea/metabolism
MH  - Stria Vascularis/cytology/metabolism
MH  - beta-Galactosidase/biosynthesis/genetics
MH  - Gap Junction beta-1 Protein
EDAT- 2011/08/05 06:00
MHDA- 2011/12/28 06:00
CRDT- 2011/08/05 06:00
PHST- 2010/09/17 00:00 [received]
PHST- 2011/04/20 00:00 [revised]
PHST- 2011/05/03 00:00 [accepted]
PHST- 2011/08/05 06:00 [entrez]
PHST- 2011/08/05 06:00 [pubmed]
PHST- 2011/12/28 06:00 [medline]
AID - S0171-9335(11)00098-7 [pii]
AID - 10.1016/j.ejcb.2011.05.001 [doi]
PST - ppublish
SO  - Eur J Cell Biol. 2011 Oct;90(10):817-24. doi: 10.1016/j.ejcb.2011.05.001. Epub 
      2011 Aug 2.