scholarly journals The lhfpl5 ohnologs lhfpl5a and lhfpl5b are required for mechanotransduction in distinct populations of sensory hair cells in zebrafish

2019 ◽  
Author(s):  
Timothy Erickson ◽  
Itallia V. Pacentine ◽  
Alexandra Venuto ◽  
Rachel Clemens ◽  
Teresa Nicolson
Keyword(s):  
Inner Ear ◽  
Lateral Line ◽  
Hair Cells ◽  
Vestibular Function ◽  
Fusion Partner ◽  
Primary Role ◽  
Mechanical Stimuli ◽  
Vestibular Hair Cells ◽  
Lateral Line Organ ◽  
Line Organ

1AbstractHair cells sense and transmit auditory, vestibular, and hydrodynamic information by converting mechanical stimuli into electrical signals. This process of mechano-electrical transduction (MET) requires a mechanically-gated channel localized in the apical stereocilia of hair cells. In mice, lipoma HMGIC fusion partner-like 5 (LHFPL5) acts as an auxiliary subunit of the MET channel whose primary role is to correctly localize PCDH15 and TMC1 to the mechanotransduction complex. Zebrafish have two lhfpl5 genes (lhfpl5a and lhfpl5b), but their individual contributions to MET channel assembly and function have not been analyzed.Here we show that the zebrafish lhfpl5 genes are expressed in discrete populations of hair cells: lhfpl5a expression is restricted to auditory and vestibular hair cells in the inner ear, while lhfpl5b expression is specific to hair cells of the lateral line organ. Consequently, lhfpl5a mutants exhibit defects in auditory and vestibular function, while disruption of lhfpl5b affects hair cells only in the lateral line neuromasts. In contrast to previous reports in mice, localization of Tmc1 does not depend upon Lhfpl5 function in either the inner ear or lateral line organ. In both lhfpl5a and lhfpl5b mutants, GFP-tagged Tmc1 and Tmc2b proteins still localize to the stereocilia of hair cells. Using a stably integrated GFP-Lhfpl5a transgene, we show that the tip link cadherins Pcdh15a and Cdh23, along with the Myo7aa motor protein, are required for correct Lhfpl5a localization at the tips of stereocilia. Our work corroborates the evolutionarily conserved co-dependence between Lhfpl5 and Pcdh15, but also reveals novel requirements for Cdh23 and Myo7aa to correctly localize Lhfpl5a. In addition, our data suggest that targeting of Tmc1 and Tmc2b proteins to stereocilia in zebrafish hair cells occurs independently of Lhfpl5 proteins.

Extracellular Receptor Potentials from the Lateral-Line Organ of Xenopus Laevis

1980 ◽  
Vol 86 (1) ◽  
pp. 63-77
Author(s):  
ALFONS B. A. KROESE ◽  
JOHAN M. VAN DER ZALM ◽  
JOEP VAN DEN BERCKEN
Keyword(s):  
Xenopus Laevis ◽  
Lateral Line ◽  
Hair Cells ◽  
Water Displacement ◽  
Stimulus Amplitude ◽  
Nerve Fibres ◽  
Large Stimulus ◽  
Sensory Hair ◽  
Lateral Line Organ ◽  
Line Organ

1. The response of the epidermal lateral-line organ of Xenopus laevis to stimulation was studied by recording extracellular receptor potentials from the hair cells in single neuromasts in isolated preparations. One neuromast was stimulated by local, sinusoidal water movements induced by a glass sphere positioned at a short distance from the neuromast. 2. The amplitudes of the extracellular receptor potentials were proportional to the stimulus amplitude over a range of 20 dB. The phase of the extracellular receptor potentials with respect to water displacement was independent of the stimulus amplitude. 3. With large stimulus amplitude, and stimulus frequencies between 0.5 Hz and 2 Hz, the extracellular receptor potentials, and responses of single afferent nerve fibres, showed a phase lead of 1.2 π radians with respect to water displacement, i.e. they were almost in phase with water acceleration. 4. It is concluded that under conditions of stimulation with small-amplitude water movements, the hair cells respond to sensory hair displacement, whereas under conditions of stimulation with large-amplitude water movements they respond to sensory hair velocity.

scholarly journals The Mechanosensory Transduction Machinery in Inner Ear Hair Cells

2020 ◽  
Vol 50 (1) ◽  
Author(s):  
Wang Zheng ◽  
Jeffrey R. Holt
Keyword(s):  
Hair Cell ◽  
Inner Ear ◽  
Hair Cells ◽  
Annual Review ◽  
Publication Date ◽  
Fusion Partner ◽  
Mechanical Stimuli ◽  
Physical Stimulation ◽  
Mechanosensory Transduction ◽  
Protocadherin 15

Sound-induced mechanical stimuli are detected by elaborate mechanosensory transduction (MT) machinery in highly specialized hair cells of the inner ear. Genetic studies of inherited deafness in the past decades have uncovered several molecular constituents of the MT complex, and intense debate has surrounded the molecular identity of the pore-forming subunits. How the MT components function in concert in response to physical stimulation is not fully understood. In this review, we summarize and discuss multiple lines of evidence supporting the hypothesis that transmembrane channel-like 1 is a long-sought MT channel subunit. We also review specific roles of other components of the MT complex, including protocadherin 15, cadherin 23, lipoma HMGIC fusion partner-like 5, transmembrane inner ear, calcium and integrin-binding family member 2, and ankyrins. Based on these recent advances, we propose a unifying theory of hair cell MT that may reconcile most of the functional discoveries obtained to date. Finally, we discuss key questions that need to be addressed for a comprehensive understanding of hair cell MT at molecular and atomic levels. Expected final online publication date for the Annual Review of Biophysics, Volume 50 is May 6, 2021. Please see http://www.annualreviews.org/page/journal/pubdates for revised estimates.

scholarly journals Conserved and Divergent Principles of Planar Polarity Revealed by Hair Cell Development and Function

2021 ◽  
Vol 15 ◽  
Author(s):  
Michael R. Deans
Keyword(s):  
Hair Cell ◽  
Inner Ear ◽  
Hair Cells ◽  
Model Organism ◽  
Vestibular Function ◽  
Sensory Receptor ◽  
Apical Surface ◽  
Planar Polarity ◽  
Vestibular Hair Cells ◽  
Sensory Epithelia

Planar polarity describes the organization and orientation of polarized cells or cellular structures within the plane of an epithelium. The sensory receptor hair cells of the vertebrate inner ear have been recognized as a preeminent vertebrate model system for studying planar polarity and its development. This is principally because planar polarity in the inner ear is structurally and molecularly apparent and therefore easy to visualize. Inner ear planar polarity is also functionally significant because hair cells are mechanosensors stimulated by sound or motion and planar polarity underlies the mechanosensory mechanism, thereby facilitating the auditory and vestibular functions of the ear. Structurally, hair cell planar polarity is evident in the organization of a polarized bundle of actin-based protrusions from the apical surface called stereocilia that is necessary for mechanosensation and when stereociliary bundle is disrupted auditory and vestibular behavioral deficits emerge. Hair cells are distributed between six sensory epithelia within the inner ear that have evolved unique patterns of planar polarity that facilitate auditory or vestibular function. Thus, specialized adaptations of planar polarity have occurred that distinguish auditory and vestibular hair cells and will be described throughout this review. There are also three levels of planar polarity organization that can be visualized within the vertebrate inner ear. These are the intrinsic polarity of individual hair cells, the planar cell polarity or coordinated orientation of cells within the epithelia, and planar bipolarity; an organization unique to a subset of vestibular hair cells in which the stereociliary bundles are oriented in opposite directions but remain aligned along a common polarity axis. The inner ear with its complement of auditory and vestibular sensory epithelia allows these levels, and the inter-relationships between them, to be studied using a single model organism. The purpose of this review is to introduce the functional significance of planar polarity in the auditory and vestibular systems and our contemporary understanding of the developmental mechanisms associated with organizing planar polarity at these three cellular levels.

scholarly journals Polydimethylsiloxane (PDMS) Waveguide Sensor Detecting Fluid Flow Velocity by Mimicking the Fish Lateral Line Organ

Proceedings ◽  
2018 ◽  
Vol 2 (13) ◽  
pp. 885
Author(s):  
Bianca Wiesmayr ◽  
Michael Krieger ◽  
Werner Baumgartner ◽  
Anna T. Stadler
Keyword(s):  
Fluid Flow ◽  
Lateral Line ◽  
Hair Cells ◽  
Water Movement ◽  
Neural Signals ◽  
Lateral Line Organ ◽  
Fluid Flow Velocity ◽  
Light Loss ◽  
Line Organ ◽  
Waveguide Sensor

Accurate measurement of fluid flow velocities is challenging but essential in many disciplines. Inspiration of possible measurement methods can come from nature, for example from the lateral line organ of fish, which is comprised of hair cells embedded in a gelatinous cupula. When the cupula is deflected by water movement, the hair cells initiate neural signals that generate an accurate image of the fish’s surroundings. We built a flow sensor mimicking a hair cell, yet coupled it with an optical detection method. Fluid flow bends the waveguide; this leads to a measurable light loss that depends linearly on the waveguide deflection.

scholarly journals Responses to Chemical Stimuli in the Hair Cells of the Lateral-line Organ of Mudpuppy

1974 ◽  
Vol 50 (7) ◽  
pp. 526-531 ◽  
Author(s):  
Keiji YANAGISAWA ◽  
Vanni TAGLIETTI ◽  
Yasuji KATSUKI
Keyword(s):  
Lateral Line ◽  
Hair Cells ◽  
Lateral Line Organ ◽  
Chemical Stimuli ◽  
Line Organ

scholarly journals Transgenic Tmc2 expression preserves inner ear hair cells and vestibular function in mice lacking Tmc1

2018 ◽  
Vol 8 (1) ◽  
Author(s):  
Yukako Asai ◽  
Bifeng Pan ◽  
Carl Nist-Lund ◽  
Alice Galvin ◽  
Andrei N. Lukashkin ◽  
...  
Keyword(s):  
Inner Ear ◽  
Hair Cells ◽  
Vestibular Function
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