Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Gap junctions and connexin expression in the inner ear.

A Forge1, D Becker, S Casalotti

  • 1Institute of Laryngology and Otology, University College London, UK.

Novartis Foundation Symposium
|April 20, 1999
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

High-yielding repetitive somatic embryogenesis and plant recovery in a selected tea clone, 'TRI-2025', by temporary immersion.

Plant cell reports·2019
Same author

The mitochondrial type IB topoisomerase drives mitochondrial translation and carcinogenesis.

Nature communications·2019
Same author

[54-year-old male with recurrent facial dermatitis with papules and vesicles on the extremities : Preparation for the medical specialist examination: Part 26].

Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete·2018
Same author

Canine NAPEPLD-associated models of human myelin disorders.

Scientific reports·2018
Same author

Associations between participation in family activities and adolescent school problems.

Child: care, health and development·2017
Same author

Gating of the two-pore cation channel AtTPC1 in the plant vacuole is based on a single voltage-sensing domain.

Plant biology (Stuttgart, Germany)·2016

Mutations in the connexin26 (Cx26) gene disrupt ion flow in the cochlea, essential for hearing. This disruption of gap junctions leads to hereditary deafness.

Area of Science:

  • Genetics
  • Neuroscience
  • Cell Biology

Background:

  • Recessive mutations in the connexin26 (Cx26) gene are linked to hereditary deafness, highlighting the importance of gap junctions in cochlear function.
  • Large gap junctions are abundant between supporting cells in the inner ear's vestibular and auditory epithelia.
  • Cx26 is the primary connexin in the cochlea's organ of Corti, with Cx32 and Cx43 absent.

Purpose of the Study:

  • To investigate the role of connexin26 (Cx26) and gap junctions in cochlear function and hereditary deafness.
  • To explore the relationship between gap junctions, ion transport, and auditory acuity in the cochlea.

Main Methods:

  • Immunohistochemistry to detect connexin isoforms (Cx26, Cx32, Cx43) in the cochlea and vestibular organs.
  • Analysis of gap junction distribution and expression in relation to cochlear development and endocochlear potential.

Related Experiment Videos

Main Results:

  • Cx26 is highly expressed in vestibular supporting cells, alongside Cx32.
  • In the cochlea, Cx26 is the predominant connexin in the organ of Corti, with Cx32 and Cx43 undetectable.
  • Gap junctions are found between supporting cells, stria vascularis basal cells, and spiral ligament fibrocytes, coinciding with endocochlear potential development.

Conclusions:

  • Gap junctions, particularly those involving Cx26, facilitate ion transport crucial for maintaining endolymph and auditory sensitivity.
  • Disruption of Cx26 function via mutations can impair ion circulation, leading to non-syndromic hereditary deafness.