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auditory receptor cell stereocilium organization

Definition

Target type: biologicalprocess

A process that is carried out at the cellular level which results in the assembly, arrangement of constituent parts, or disassembly of a stereocilium. A stereocilium is an actin-based protrusion from the apical surface of auditory hair cells. [GOC:dph, PMID:10978835]

Auditory receptor cells, also known as hair cells, are specialized sensory cells responsible for detecting sound vibrations. These cells possess hair-like structures called stereocilia, which are essential for transducing sound into electrical signals. The organization and arrangement of stereocilia are critical for auditory function, contributing to sensitivity, frequency tuning, and directional hearing.

The development and organization of stereocilia involve a complex interplay of various molecular and cellular processes:

**1. Formation and Elongation:**

* Stereocilia initiate their formation as actin-filled protrusions from the apical surface of hair cells.
* The core of each stereocilium is composed of a tightly bundled array of actin filaments, which are cross-linked by various proteins, including fascin and espin.
* Elongation of stereocilia occurs through the addition of new actin monomers at the tips, driven by the activity of formins, a class of actin-nucleating proteins.

**2. Stereocilia Bundling:**

* Stereocilia within a hair cell are organized into bundles, with rows of increasing height.
* The arrangement of stereocilia bundles varies across different species and within different hair cell types.
* In mammals, a typical hair cell bundle consists of three rows: a single row of tall stereocilia (the tallest), a middle row of intermediate-height stereocilia, and a row of short stereocilia.
* The arrangement of stereocilia within a bundle is maintained by tip links, which are fine, elastic filaments connecting the tips of adjacent stereocilia.
* Tip links play a crucial role in mechanotransduction, allowing the stereocilia to respond to sound vibrations.

**3. Stereocilia Orientation and Polarity:**

* The orientation of stereocilia within a bundle is crucial for directional hearing.
* The orientation of the tallest row of stereocilia (the kinocilium) defines the hair cell's polarity.
* Sound waves that deflect the stereocilia in the direction of the kinocilium (towards the taller stereocilia) cause depolarization of the hair cell, leading to auditory signal transmission.

**4. Stereocilia Maintenance and Turnover:**

* Stereocilia are constantly remodeled and maintained throughout the life of the hair cell.
* The tips of stereocilia are continuously recycled, with older actin filaments being degraded and replaced with new ones.
* This dynamic turnover process ensures the stability and integrity of the stereocilia, contributing to long-term auditory function.

**5. Role of Molecular Components:**

* A vast array of proteins are involved in the organization, function, and maintenance of stereocilia.
* These include actin-binding proteins, such as fascin and espin, which cross-link actin filaments and contribute to stereocilia structure and stability.
* Other proteins, such as cadherins and protocadherins, mediate cell-cell adhesion and contribute to the formation of stereocilia bundles.
* Mutations in genes encoding these proteins can lead to hearing loss, highlighting the importance of these molecules for auditory function.'
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Proteins (2)

ProteinDefinitionTaxonomy
Superoxide dismutase [Cu-Zn]A superoxide dismutase [Cu-Zn] that is encoded in the genome of human. [PRO:DNx, UniProtKB:P00441]Homo sapiens (human)
Na(+)/H(+) exchange regulatory cofactor NHE-RF1A Na(+)/H(+) exchange regulatory cofactor NHE-RF1 that is encoded in the genome of human. [PRO:CNA, UniProtKB:O14745]Homo sapiens (human)

Compounds (2)

CompoundDefinitionClassesRoles
formylmethionyl-leucyl-phenylalanine methyl esterpeptide
fh535FH535: inhibits Wnt signalingsulfonamide