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 Concept Videos

Hearing01:31

Hearing

51.8K
When we hear a sound, our nervous system is detecting sound waves—pressure waves of mechanical energy traveling through a medium. The frequency of the wave is perceived as pitch, while the amplitude is perceived as loudness.
51.8K
Mutations01:39

Mutations

78.5K
Overview
78.5K
Translation01:31

Translation

141.4K
Lesson: Translation
Translation is the process of synthesizing proteins from the genetic information carried by messenger RNA (mRNA). Following transcription, it constitutes the final step in the expression of genes. This process is carried out by ribosomes, complexes of protein and specialized RNA molecules. Ribosomes, transfer RNA (tRNA), and other proteins produce a chain of amino acids—the polypeptide—as the end product of translation.
Translation Produces the Building Blocks of...
141.4K

You might also read

Related Articles

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

Sort by
Same author

Robust tauopathy and memory deficits in a mouse model constitutively overexpressing human P301L MAPT.

Neurobiology of disease·2026
Same author

Mitofusins are required for specialized mitochondrial morphology and function of rod photoreceptor cells.

Frontiers in cell and developmental biology·2026
Same author

Tonotopic specialization of MYO7A isoforms in auditory hair cells.

Nature communications·2026
Same author

AAV2 Crosslinks Actin Filaments: Implications for AAV Gene Therapy Vector Design.

bioRxiv : the preprint server for biology·2026
Same author

Alveolar Type 2 Cell Dysfunction Is Associated with Bile Acid Alterations in Experimental Hepatopulmonary Syndrome.

American journal of respiratory cell and molecular biology·2026
Same author

IRE1/FAM134B-mediated ER-phagy alleviates zearalenone-induced ER stress and developmental defects in porcine embryos.

Ecotoxicology and environmental safety·2026

Related Experiment Video

Updated: May 27, 2025

Data Acquisition and Analysis In Brainstem Evoked Response Audiometry In Mice
08:51

Data Acquisition and Analysis In Brainstem Evoked Response Audiometry In Mice

Published on: May 10, 2019

11.6K

A mutation in Tmem135 causes progressive sensorineural hearing loss.

Mi-Jung Kim1, Shion Simms1, Ghazaleh Behnammanesh2

  • 1Department of Physiology and Aging, University of Florida, Gainesville, FL, United States.

Hearing Research
|February 19, 2025
PubMed
Summary

Transmembrane protein 135 (TMEM135) mutation causes progressive hearing loss in mice. TMEM135 is vital for maintaining cochlear cell function and auditory sensory integrity.

Keywords:
AgingCochleaHair cellHearing lossMitochondriaMitochondrial dynamicsTMEM135

More Related Videos

Dextran Labeling and Uptake in Live and Functional Murine Cochlear Hair Cells
05:55

Dextran Labeling and Uptake in Live and Functional Murine Cochlear Hair Cells

Published on: February 8, 2020

7.3K
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
09:44

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

Published on: January 25, 2016

19.1K

Related Experiment Videos

Last Updated: May 27, 2025

Data Acquisition and Analysis In Brainstem Evoked Response Audiometry In Mice
08:51

Data Acquisition and Analysis In Brainstem Evoked Response Audiometry In Mice

Published on: May 10, 2019

11.6K
Dextran Labeling and Uptake in Live and Functional Murine Cochlear Hair Cells
05:55

Dextran Labeling and Uptake in Live and Functional Murine Cochlear Hair Cells

Published on: February 8, 2020

7.3K
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
09:44

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

Published on: January 25, 2016

19.1K

Area of Science:

  • Genetics
  • Otolaryngology
  • Cell Biology

Background:

  • Transmembrane protein 135 (TMEM135) is a conserved protein involved in mitochondrial and peroxisomal functions.
  • Previous research links TMEM135 to metabolic processes, but its role in the auditory system is unexplored.

Purpose of the Study:

  • To investigate the function of TMEM135 in the mammalian auditory system.
  • To determine the impact of Tmem135 mutations on hearing and cochlear structure.

Main Methods:

  • Utilized wild-type and Tmem135 mutant (FUN025) mice on a CBA/CaJ background.
  • Assessed auditory function using auditory brainstem response (ABR) testing at various frequencies and ages.
  • Examined cochlear histology, including hair cell survival, spiral ganglion neuron density, and stria vascularis integrity.
  • Analyzed TMEM135 expression patterns via BaseScope RNA in situ hybridization.
  • Investigated TMEM135 colocalization with mitochondria in hair cells using Volocity and Costes analysis.

Main Results:

  • Tmem135 FUN025 mutant mice developed progressive sensorineural hearing loss starting by 3 months and becoming profound by 12 months.
  • Significant loss of outer hair cells and moderate changes in inner hair cells, spiral ganglion neurons, and stria vascularis were observed in mutant mice.
  • TMEM135 expression was detected in key cochlear cell types: inner hair cells, outer hair cells, supporting cells, and stria vascularis.
  • TMEM135 was found to colocalize with mitochondria within hair cells.

Conclusions:

  • The FUN025 mutation in Tmem135 leads to age-dependent progressive hearing impairment.
  • TMEM135 plays a critical role in the survival and function of cochlear cells, particularly hair cells.
  • TMEM135 is essential for maintaining auditory sensory function throughout the aging process.