Stereocilin connects outer hair cell stereocilia to one another and to the tectorial membrane

Verpy, Elisabeth; Leibovici, Michel; Michalski, Nicolas; Goodyear, Richard J.; Houdon, Carine; Weil, Dominique; Richardson, Guy P.; Petit, Christine

doi:10.1002/cne.22509

Cited by 105 publications

(112 citation statements)

References 34 publications

(60 reference statements)

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“…This might explain observations that loss of top connectors substantially affects cochlear non-linearity (Verpy et al, 2011). It has also been reported that the top connectors may function to reduce viscous drag on the bundle (Kozlov et al, 2011).…”

Section: Lateral Linksmentioning

confidence: 77%

“…The contact region is shown, an area that may coincide with a region of 'top connectors' but in mammals also appears to label with antibodies to amiloride sensitivity sodium channels (Hackney et al, 1992). Another region of top connectors is the lateral links at the tip of the tallest stereocilia, some of which extend into the overlaying tectorial membrane (Verpy et al, 2011). Scale bars: A53 mm; B5600 nm; C550 nm; D5100 nm; E and F5250 nm.…”

Section: Deflectionmentioning

confidence: 99%

“…The precise location and appearance of these links differs in different species and types of hair cells (see review by Furness and Hackney, 2006). Nevertheless, there are particular regions of horizontal connections (top connectors) between the tops of adjacent tall stereocilia (another category of top connectors occurs just below the tips of shorter stereocilia) and close to the lower tip link insertion (Verpy et al, 2011). Occasionally a region of close approach (the contact region) between the short and taller stereocilia is seen in this location ( Fig.…”

Section: Lateral Linksmentioning

confidence: 99%

“…There are a number of candidate molecules, although more might yet be discovered. Among the lateral links, a subset of top connectors has been found in mammalian hair cells of the organ of Corti, the outer hair cells (OHC), that contain the protein stereocilin, which was first described in a search for candidate deafness genes in mice that are linked to the human deafness locus DFNB16 (Verpy et al, 2001;Verpy et al, 2011). Stereocilin is a member of the mesothelin protein superfamily, which contains proteins that have been suggested to function as superhelical lectins that bind to carbohydrate moieties of extracellular glycoproteins (Sathyanarayana et al, 2009).…”

Section: Lateral Linksmentioning

confidence: 99%

“…It is encoded by the STRC gene, which is defective in the human deafness type DFNB16 (Verpy et al, 2001). DFNB16 is an autosomal recessive progressive condition with hearing impairment that appears in childhood and which, in mouse models, results in a lack of cohesiveness in the hair bundle after P9 (Verpy et al, 2011).…”

Section: Box 4 Hearing Loss Caused By Gene Defectsmentioning

confidence: 99%

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The composition and role of cross links in mechanoelectrical transduction in vertebrate sensory hair cells

Hackney

Furness

2013

Journal of Cell Science

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SummaryThe key components of acousticolateralis systems (lateral line, hearing and balance) are sensory hair cells. At their apex, these cells have a bundle of specialized cellular protrusions, which are modified actin-containing microvilli, connected together by extracellular filaments called cross links. Stereociliary deflections open nonselective cation channels allowing ions from the extracellular environment into the cell, a process called mechanoelectrical transduction. This produces a receptor potential that causes the release of the excitatory neurotransmitter glutamate onto the terminals of the sensory nerve fibres, which connect to the cell base, causing nerve signals to be sent to the brain. Identification of the cellular mechanisms underlying mechanoelectrical transduction and of some of the proteins involved has been assisted by research into the genetics of deafness, molecular biology and mechanical measurements of function. It is thought that one type of cross link, the tip link, is composed of cadherin 23 and protocadherin 15, and gates the transduction channel when the bundle is deflected. Another type of link, called lateral (or horizontal) links, maintains optimal bundle cohesion and stiffness for transduction. This Commentary summarizes the information currently available about the structure, function and composition of the links and how they might be relevant to human hearing impairment.

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Section: Lateral Linksmentioning

confidence: 77%

Section: Deflectionmentioning

confidence: 99%

Section: Lateral Linksmentioning

confidence: 99%

Section: Lateral Linksmentioning

confidence: 99%

Section: Box 4 Hearing Loss Caused By Gene Defectsmentioning

confidence: 99%

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The composition and role of cross links in mechanoelectrical transduction in vertebrate sensory hair cells

Hackney

Furness

2013

Journal of Cell Science

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Genome‐wide SNP genotyping identifies the Stereocilin (STRC) gene as a major contributor to pediatric bilateral sensorineural hearing impairment

Francey

Conlin

Kadesch

et al. 2011

American J of Med Genetics Pt A

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Hearing loss is the most prevalent sensory perception deficit in humans, affecting 1/500 newborns, can be syndromic or nonsyndromic and is genetically heterogeneous. Nearly 80% of inherited nonsyndromic bilateral sensorineural hearing loss (NBSNHI) is autosomal recessive. Although many causal genes have been identified, most are minor contributors, except for GJB2, which accounts for nearly 50% of all recessive cases of severe to profound congenital NBSNHI in some populations. More than 60% of children with a NBSNHI do not have an identifiable genetic cause. To identify genetic contributors, we genotyped 659 GJB2 mutation negative pediatric probands with NBSNHI and assayed for copy number variants (CNVs). After identifying 8 mild-moderate NBSNHI probands with a Chr15q15.3 deletion encompassing the Stereocilin (STRC) gene amongst this cohort, sequencing of STRC was undertaken in these probands as well as 50 probands and 14 siblings with mild-moderate NBSNHI and 40 probands with moderately severe-profound NBSNHI who were GJB2 mutation negative. The existence of a STRC pseudogene that is 99.6% homologous to the STRC coding region has made the sequencing interpretation complicated. We identified 7/50 probands in the mild-moderate cohort to have biallelic alterations in STRC, not including the 8 previously identified deletions. We also identified 2/40 probands to have biallelic alterations in the moderately severe-profound NBSNHI cohort, notably no large deletions in combination with another variant were found in this cohort. The data suggest that STRC may be a common contributor to NBSNHI among GJB2 mutation negative probands, especially in those with mild to moderate hearing impairment.

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Exorhodopsin and melanopsin systems in the pineal complex and brain at early developmental stages of Atlantic halibut (Hippoglossus hippoglossus)

Eilertsen

Drivenes

Edvardsen

et al. 2014

J of Comparative Neurology

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The complexity of the nonvisual photoreception systems in teleosts has just started to be appreciated, with colocalization of multiple photoreceptor types with unresolved functions. Here we describe an intricate expression pattern of melanopsins in early life stages of the marine flat fish Atlantic halibut (Hippoglossus hippoglossus), a period when the unpigmented brain is directly exposed to environmental photons. We show a refined and extensive expression of melanopsins in the halibut brain already at the time of hatching, long before the eyes are functional. We detect melanopsin in the habenula, suprachiasmatic nucleus, dorsal thalamus, and lateral tubular nucleus of first feeding larvae, suggesting conserved functions of the melanopsins in marine teleosts. The complex expression of melanopsins already at larval stages indicates the importance of nonvisual photoreception early in development. Most strikingly, we detect expression of both exorhodopsin and melanopsin in the pineal complex of halibut larvae. Double-fluorescence labeling showed that two clusters of melanopsin-positive cells are located lateral to the central rosette of exorhodopsin-positive cells. The localization of different photopigments in the pineal complex suggests that two parallel photoreceptor systems may be active. Furthermore, the dispersed melanopsin-positive cells in the spinal cord of halibut larvae at the time of hatching may be primary sensory cells or interneurons representing the first example of dispersed high-order photoreceptor cells. The appearance of nonvisual opsins early in the development of halibut provides an alternative model for studying the evolution and functional significance of nonvisual opsins.

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Stereocilin connects outer hair cell stereocilia to one another and to the tectorial membrane

Cited by 105 publications

References 34 publications

The composition and role of cross links in mechanoelectrical transduction in vertebrate sensory hair cells

The composition and role of cross links in mechanoelectrical transduction in vertebrate sensory hair cells

Genome‐wide SNP genotyping identifies the Stereocilin (STRC) gene as a major contributor to pediatric bilateral sensorineural hearing impairment

Exorhodopsin and melanopsin systems in the pineal complex and brain at early developmental stages of Atlantic halibut (Hippoglossus hippoglossus)

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