|
Tip-link
Tip links are extracellular filaments that connect stereocilia to each other or to the kinocilium in the hair cells of the inner ear.Pickles JO, Comis SD, Osborne MP. 1984.Cross-links between stereocilia in the guinea pig organ of Corti, and their possible relation to sensory transduction. Hearing Research 15:103-112. Mechanotransduction is thought to occur at the site of the tip links, which connect to spring-gated ion channels. These channels are cation-selective transduction channels that allow potassium and calcium ions to enter the hair cell from the endolymph that bathes its apical end. When the hair cells are deflected toward the kinocilium, depolarization occurs; when deflection is away from the kinocilium, hyperpolarization occurs. The tip link is made of two different cadherin molecules, protocadherin 15 and cadherin 23.Lewin GR, Moshourab R. 2004. Mechanosensation and pain. Journal of Neurobiology 61:30-44 It has been found that the tip links are relatively stiff, so it ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
CDH23
Cadherin-23 is a protein that in humans is encoded by the ''CDH23'' gene. Function This gene is a member of the cadherin superfamily, genes encoding calcium dependent cell-cell adhesion glycoproteins. The protein encoded by this gene is a large, single-pass transmembrane protein composed of an extracellular domain containing 27 repeats that show significant homology to the cadherin ectodomain. Expressed in the neurosensory epithelium, the protein is thought to be involved in stereocilia organization and hair bundle formation. Specifically, it is thought to interact with protocadherin 15 to form tip-link filaments. Clinical significance The gene is located in a region containing the human deafness loci DFNB12 and USH1D. Usher syndrome 1D and nonsyndromic autosomal recessive deafness DFNB12 are caused by allelic mutations of this novel cadherin-like gene. The gene is associated with kidney In humans, the kidneys are two reddish-brown bean-shaped blood-filtering organ ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Protocadherin 15
Protocadherin-15 is a protein that in humans is encoded by the ''PCDH15'' gene. Function This gene is a member of the cadherin superfamily. Family members encode integral membrane proteins that mediate calcium-dependent cell-cell adhesion. The protein product of this gene consists of a signal peptide, 11 extracellular calcium-binding domains, a transmembrane domain and a unique cytoplasmic domain. It plays an essential role in maintenance of normal retinal and cochlear function. It is thought to interact with CDH23 to form tip-link filaments. Clinical significance Mutations in this gene have been associated with hearing loss, which is consistent with its location at the Usher syndrome Usher syndrome, also known as Hallgren syndrome, Usher–Hallgren syndrome, retinitis pigmentosa–dysacusis syndrome or dystrophia retinae dysacusis syndrome, is a rare genetic disorder caused by a mutation in any one of at least 11 genes result ... type 1F (USH1F) critical region on chrom ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
The Tailchaser Mutation Does Not Affect Formation Of Interstereocilial Links
''The'' is a grammatical article in English, denoting nouns that are already or about to be mentioned, under discussion, implied or otherwise presumed familiar to listeners, readers, or speakers. It is the definite article in English. ''The'' is the most frequently used word in the English language; studies and analyses of texts have found it to account for seven percent of all printed English-language words. It is derived from gendered articles in Old English which combined in Middle English and now has a single form used with nouns of any gender. The word can be used with both singular and plural nouns, and with a noun that starts with any letter. This is different from many other languages, which have different forms of the definite article for different genders or numbers. Pronunciation In most dialects, "the" is pronounced as (with the voiced dental fricative followed by a schwa) when followed by a consonant sound, and as (homophone of the archaic pronoun ''thee' ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Stereocilia
Stereocilia (or stereovilli or villi) are non-motile apical cell modifications. They are distinct from cilia and microvilli, but are closely related to microvilli. They form single "finger-like" projections that may be branched, with normal cell membrane characteristics. They contain actin. Stereocilia are found in the vas deferens, the epididymis, and the sensory cells of the inner ear. Structure Stereocilia are cylindrical and non-motile. They are much longer and thicker than microvilli, form single "finger-like" projections that may be branched, and have more of the characteristics of the cellular membrane proper. Like microvilli, they contain actin and lack an axoneme. This distinguishes them from cilia. They do not have a basal body at their base since they do not contain microtubules. They may or may not be covered by a glycocalyx coating. They have no fixed arrangement, different to the structure present in kinocilium. Organs containing stereocilia Stereocilia are ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Kinocilium
A kinocilium is a special type of cilium on the apex of hair cells located in the sensory epithelium of the vertebrate inner ear. Contrasting with stereocilia, which are numerous, there is only one kinocilium on each hair cell. The kinocilium can be identified by its apical position as well as its enlarged tip. Together with stereocilia, the kinocilium regulates depolarization and hyperpolarization of the hair cell, which is a neuron that can generate action potentials. When the stereocilia and kinocilium move further apart, the cell hyperpolarizes. When they move closer together, the cell depolarizes and may fire an action potential. Anatomy in humans Kinocilia are found on the apical surface of hair cells and are involved in both the morphogenesis of the hair bundle and mechanotransduction. Vibrations (either by movement or sound waves) cause displacement of the hair bundle, resulting in depolarization or hyperpolarization of the hair cell. The depolarization of the hair cells ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Inner Ear
The inner ear (internal ear, auris interna) is the innermost part of the vertebrate ear. In vertebrates, the inner ear is mainly responsible for sound detection and balance. In mammals, it consists of the bony labyrinth, a hollow cavity in the temporal bone of the skull with a system of passages comprising two main functional parts: * The cochlea, dedicated to hearing; converting sound pressure patterns from the outer ear into electrochemical impulses which are passed on to the brain via the auditory nerve. * The vestibular system, dedicated to balance (ability), balance. The inner ear is found in all vertebrates, with substantial variations in form and function. The inner ear is innervated by the eighth cranial nerve in all vertebrates. Structure The labyrinth can be divided by layer or by region. Bony and membranous labyrinths The bony labyrinth, or osseous labyrinth, is the network of passages with bony walls lined with periosteum. The three major parts of the bony labyrin ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Mechanotransduction
In cellular biology, mechanotransduction ('' mechano'' + '' transduction'') is any of various mechanisms by which cells convert mechanical stimulus into electrochemical activity. This form of sensory transduction is responsible for a number of senses and physiological processes in the body, including proprioception, touch, balance, and hearing. The basic mechanism of mechanotransduction involves converting mechanical signals into electrical or chemical signals. In this process, a mechanically gated ion channel makes it possible for sound, pressure, or movement to cause a change in the excitability of specialized sensory cells and sensory neurons. The stimulation of a mechanoreceptor causes mechanically sensitive ion channels to open and produce a transduction current that changes the membrane potential of the cell. Typically the mechanical stimulus gets filtered in the conveying medium before reaching the site of mechanotransduction. Cellular responses to mechanotransducti ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Endolymph
Endolymph is the fluid contained in the membranous labyrinth of the inner ear. The major cation in endolymph is potassium, with the values of sodium and potassium concentration in the endolymph being 0.91 mM and 154 mM, respectively. It is also called ''Scarpa's fluid'', after Antonio Scarpa. Structure The inner ear has two parts: the bony labyrinth and the membranous labyrinth. The membranous labyrinth is contained within the bony labyrinth, and within the membranous labyrinth is a fluid called endolymph. Between the outer wall of the membranous labyrinth and the wall of the bony labyrinth is the location of perilymph. Composition Perilymph and endolymph have unique ionic compositions suited to their functions in regulating electrochemical impulses of hair cells. The electric potential of endolymph is ~80-90 mV more positive than perilymph due to a higher concentration of K compared to Na. The main component of this unique extracellular fluid is potassium, whic ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Cadherin
Cadherins (named for "calcium-dependent adhesion") are cell adhesion molecules important in forming adherens junctions that let cells adhere to each other. Cadherins are a class of type-1 transmembrane proteins, and they depend on calcium (Ca2+) ions to function, hence their name. Cell-cell adhesion is mediated by extracellular cadherin domains, whereas the Cadherin cytoplasmic region, intracellular cytoplasmic tail associates with numerous adaptors and signaling proteins, collectively referred to as the cadherin adhesome. Background The cadherin family is essential in maintaining cell-cell contact and regulating cytoskeletal complexes. The cadherin superfamily includes cadherins, protocadherins, desmogleins, desmocollins, and more. In structure, they share ''cadherin repeats'', which are the extracellular Ca2+-binding domains. There are multiple classes of cadherin molecules, each designated with a prefix for tissues with which it associates. Classical cadherins maintain the ton ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Actin
Actin is a family of globular multi-functional proteins that form microfilaments in the cytoskeleton, and the thin filaments in muscle fibrils. It is found in essentially all eukaryotic cells, where it may be present at a concentration of over 100 μM; its mass is roughly 42 kDa, with a diameter of 4 to 7 nm. An actin protein is the monomeric subunit of two types of filaments in cells: microfilaments, one of the three major components of the cytoskeleton, and thin filaments, part of the contractile apparatus in muscle cells. It can be present as either a free monomer called G-actin (globular) or as part of a linear polymer microfilament called F-actin (filamentous), both of which are essential for such important cellular functions as the mobility and contraction of cells during cell division. Actin participates in many important cellular processes, including muscle contraction, cell motility, cell division and cytokinesis, vesicle and organelle mov ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Neuronal Encoding Of Sound
A neuron (American English), neurone (British English), or nerve cell, is an excitable cell that fires electric signals called action potentials across a neural network in the nervous system. They are located in the nervous system and help to receive and conduct impulses. Neurons communicate with other cells via synapses, which are specialized connections that commonly use minute amounts of chemical neurotransmitters to pass the electric signal from the presynaptic neuron to the target cell through the synaptic gap. Neurons are the main components of nervous tissue in all animals except sponges and placozoans. Plants and fungi do not have nerve cells. Molecular evidence suggests that the ability to generate electric signals first appeared in evolution some 700 to 800 million years ago, during the Tonian period. Predecessors of neurons were the peptidergic secretory cells. They eventually gained new gene modules which enabled cells to create post-synaptic scaffolds and ion cha ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Vestibular System
The vestibular system, in vertebrates, is a sensory system that creates the sense of balance and spatial orientation for the purpose of coordinating motor coordination, movement with balance. Together with the cochlea, a part of the auditory system, it constitutes the labyrinth of the inner ear in most Mammal, mammals. As movements consist of rotations and translations, the vestibular system comprises two components: the semicircular canals, which indicate Angular acceleration, rotational movements; and the otoliths, which indicate Acceleration, linear accelerations. The vestibular system sends signals primarily to the neural structures that control eye movement; these provide the anatomical basis of the vestibulo-ocular reflex, which is required for clear vision. Signals are also sent to the muscles that keep an animal upright and in general control Neutral spine, posture; these provide the anatomical means required to enable an animal to maintain its desired position in space. ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
