Navigable Slide Index
- Lecture overview
- Auditory pathway
- Ion channels initiate sound transmission
- Voltage-gated Ca2+ channels trigger signaling
- Voltage-gated Ca2+ channels
- Measurements of Ca2+ currents (1)
- Measurements of Ca2+ currents (2)
- Which channels expressed in inner hair cells?
- Cav1.3 the primary Cav in mouse inner hair cells
- Hearing is affected without Cav1.3 channels
- Mice lacking Cav1.3 are deaf
- Loss of Cav1.3 also causes cardiac arrhythmia
- LOF of Cav1.3 causes deafness in humans
- Deafness-causing mutation in CACNA1D
- Congenital hearing loss in homozygous carriers
- CACNA1D mutation causes cardiac arrhythmia
- How does CACNA1D mutation cause deafness?
- Glycine insertion occurs in a conserved region
- Testing the functional effects of the insertion
- Glycine insertion blocks Cav1.3 function
- Why are mutant channels non-functional?
- Measuring gating currents
- Mutant channels do not conduct Ca2+ ions
- Cav1.3 LOF causes deafness in mice and humans
- What about Cav1.3 gain-of-function?
- The harmonin gene
- Cav1.3 interacts with PDZ-proteins
- Harmonin binds to Cav1.3
- The functional effects of harmonin on Cav1.3
- Harmonin inhibits Cav1.3 current density
- Harmonin regulate synaptic Cav1.3 channels
- dfcr-harmonin does not interact with Cav1.3
- Harmonin regulate Cav1.3 in inner hair cells
- Harmonin inhibits Cav1.3 currents
- How does harmonin inhibit Cav1.3?
- Proteosomal degradation controls protein levels
- MG132 reverses harmonin inhibition of Cav1.3
- Regulation of Cav1.3 by harmonin at synapse
- Function of Cav1.3 is necessary for hearing
- Auditory transmission
- Voltage-gated Ca(v) Ca2+ channels
- Role of Ca(v)1.3 in auditory hair cells
- How alterations in Ca(v)1.3 may cause deafness
Talk CitationLee, A. (2012, October 31). Ca(v)1.3 and deafness [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved May 29, 2023, from https://hstalks.com/bs/2413/.
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- Dr. Amy Lee has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.
A selection of talks on Biochemistry