Immunolocalization of connexin 26 in the developing mouse cochlea

Frenz, Christopher M.; Water, Thomas R. Van De

doi:10.1016/s0165-0173(99)00078-8

Cited by 24 publications

(16 citation statements)

References 26 publications

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“…At the onset of hearing, Cx26 and Cx30 expressions in the lateral wall quickly changes from primarily a band of cells bordering the stria vascularis to almost all the cells above the spiral ligament. Studies agree that Cx26 and Cx30 are not expressed in both inner and outer hair cells, nor in marginal cells of the stria vascularis (Ahmad et al, 2003; Forge et al, 2003a; Frenz and Van De Water, 2000; Kikuchi et al, 1995; Lautermann et al, 1998; Liu and Zhao, 2008; Zhao and Yu, 2006). Whether intermediate cells of the stria vascularis express functional level of Cx26 and Cx30 is still controversial.…”

Section: Molecular Structural Basis Of Gj Network In the Cochleamentioning

confidence: 84%

Diverse deafness mechanisms of connexin mutations revealed by studies using in vitro approaches and mouse models

et al. 2009

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Mutations in connexins (Cxs), the constitutive protein subunits of gap junction (GJ) intercellular channels, are one of the most common human genetic defects that cause severe prelingual non-syndromic hearing impairments. Many subtypes of Cxs (e.g., Cxs 26, 29, 30, 31, 43) and pannexins (Panxs) are expressed in the cochlea where they contribute to the formation of a GJ-based intercellular communication network. Cx26 and Cx30 are the predominant cochlear Cxs and they co-assemble in most GJ plaques to form hybrid GJs. The cellular localization of specific Cx subtypes provides a basis for understanding the molecular structure of GJs and hemichannels in the cochlea. Information about the interactions among the various co-assembled Cx partners is critical to appreciate the functional consequences of various types of genetic mutations. In vitro studies of reconstituted GJs in cell lines have yielded surprisingly heterogeneous mechanisms of dysfunction caused by various Cx mutations. Availability of multiple lines of Cx-mutant mouse models has provided some insight into the pathogenesis processes in the cochlea of deaf mice. Here we summarize recent advances in understanding the structure and function of cochlear GJs and give a critical review of current findings obtained from both in vitro studies and mouse models on the mechanisms of Cx mutations that lead to cell death in the cochlea and hearing loss.

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Section: Molecular Structural Basis Of Gj Network In the Cochleamentioning

confidence: 84%

Diverse deafness mechanisms of connexin mutations revealed by studies using in vitro approaches and mouse models

et al. 2009

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“…In rodents, several connexins (Cx26, Cx30, Cx31, Cx32, and Cx43) have been detected in the epithelia and connective tissue of the cochlea (15,32,36,47,55,56,60,104,112), supporting the formation of a gap-junction network in these tissues. In addition, gapjunction plaques (32-35, 50, 51) and extensive intercellular coupling (4, 35, 64, 84-86, 110, 111) were observed in both the cochlear connective tissue and the organ of Corti.…”

Section: Connexin Expression In Inner Ear and Gap-junction Functionmentioning

confidence: 92%

Gap-Junction Channels Dysfunction in Deafness and Hearing Loss

Martı́nez

Acuña

Figueroa

et al. 2009

Antioxidants & Redox Signaling

132

107

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Gap-junction channels connect the cytoplasm of adjacent cells, allowing the diffusion of ions and small metabolites. They are formed at the appositional plasma membranes by a family of related proteins named connexins. Mutations in connexins 26, 31, 30, 32, and 43 have been associated with nonsyndromic or syndromic deafness. The majority of these mutations are inherited in an autosomal recessive manner, but a few of them have been associated with dominantly inherited hearing loss. Mutations in the connexin26 gene (GJB2) are the most common cause of genetic deafness. This review summarizes the most relevant and recent information about different mutations in connexin genes found in human patients, with emphasis on GJB2. The possible effects of the mutations on channel expression and function are discussed, in addition to their possible physiologic consequences for inner ear physiology. Finally, we propose that connexin channels (gap junctions and hemichannels) may be targets for age-related hearing loss induced by oxidative damage. Antioxid. Redox Signal. 11, 309-322. 309

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“…Connexins are expressed in the epithelia and connective tissues, but not in the sensory cells (the hair cells) of the cochlea. They establish a functional syncytium among these cells (183, 188). In the cochlea, Cx26 and Cx32 may be involved in buffering and recycling of K + (183,418).…”

Section: Physiological Function In Cell-to-cell Communicationmentioning

confidence: 99%

Gap Junctions

Nielsen,

Nygaard Axelsen,

Sorgen

et al. 2012

Comprehensive Physiology

373

365

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Gap junctions are essential to the function of multicellular animals, which require a high degree of coordination between cells. In vertebrates, gap junctions comprise connexins and currently 21 connexins are known in humans. The functions of gap junctions are highly diverse and include exchange of metabolites and electrical signals between cells, as well as functions, which are apparently unrelated to intercellular communication. Given the diversity of gap junction physiology, regulation of gap junction activity is complex. The structure of the various connexins is known to some extent; and structural rearrangements and intramolecular interactions are important for regulation of channel function. Intercellular coupling is further regulated by the number and activity of channels present in gap junctional plaques. The number of connexins in cell‐cell channels is regulated by controlling transcription, translation, trafficking, and degradation; and all of these processes are under strict control. Once in the membrane, channel activity is determined by the conductive properties of the connexin involved, which can be regulated by voltage and chemical gating, as well as a large number of posttranslational modifications. The aim of the present article is to review our current knowledge on the structure, regulation, function, and pharmacology of gap junctions. This will be supported by examples of how different connexins and their regulation act in concert to achieve appropriate physiological control, and how disturbances of connexin function can lead to disease. © 2012 American Physiological Society. Compr Physiol 2:1981‐2035, 2012.

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Immunolocalization of connexin 26 in the developing mouse cochlea

Cited by 24 publications

References 26 publications

Diverse deafness mechanisms of connexin mutations revealed by studies using in vitro approaches and mouse models

Diverse deafness mechanisms of connexin mutations revealed by studies using in vitro approaches and mouse models

Gap-Junction Channels Dysfunction in Deafness and Hearing Loss

Gap Junctions

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