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Letteratura scientifica selezionata sul tema "Stereocilin"

Autore: Grafiati

Pubblicato: 7 settembre 2024

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Articoli di riviste sul tema "Stereocilin"

Avan, Paul, Sébastien Le Gal, Vincent Michel, Typhaine Dupont, Jean-Pierre Hardelin, Christine Petit e Elisabeth Verpy. "Otogelin, otogelin-like, and stereocilin form links connecting outer hair cell stereocilia to each other and the tectorial membrane". Proceedings of the National Academy of Sciences 116, n. 51 (27 novembre 2019): 25948–57. http://dx.doi.org/10.1073/pnas.1902781116.

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The function of outer hair cells (OHCs), the mechanical actuators of the cochlea, involves the anchoring of their tallest stereocilia in the tectorial membrane (TM), an acellular structure overlying the sensory epithelium. Otogelin and otogelin-like are TM proteins related to secreted epithelial mucins. Defects in either cause the DFNB18B and DFNB84B genetic forms of deafness, respectively, both characterized by congenital mild-to-moderate hearing impairment. We show here that mutant mice lacking otogelin or otogelin-like have a marked OHC dysfunction, with almost no acoustic distortion products despite the persistence of some mechanoelectrical transduction. In both mutants, these cells lack the horizontal top connectors, which are fibrous links joining adjacent stereocilia, and the TM-attachment crowns coupling the tallest stereocilia to the TM. These defects are consistent with the previously unrecognized presence of otogelin and otogelin-like in the OHC hair bundle. The defective hair bundle cohesiveness and the absence of stereociliary imprints in the TM observed in these mice have also been observed in mutant mice lacking stereocilin, a model of the DFNB16 genetic form of deafness, also characterized by congenital mild-to-moderate hearing impairment. We show that the localizations of stereocilin, otogelin, and otogelin-like in the hair bundle are interdependent, indicating that these proteins interact to form the horizontal top connectors and the TM-attachment crowns. We therefore suggest that these 2 OHC-specific structures have shared mechanical properties mediating reaction forces to sound-induced shearing motion and contributing to the coordinated displacement of stereocilia.

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Cartagena-Rivera, Alexander X., Sébastien Le Gal, Kerianne Richards, Elisabeth Verpy e Richard S. Chadwick. "Cochlear outer hair cell horizontal top connectors mediate mature stereocilia bundle mechanics". Science Advances 5, n. 2 (febbraio 2019): eaat9934. http://dx.doi.org/10.1126/sciadv.aat9934.

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Outer hair cell (OHC) stereocilia bundle deflection opens mechanoelectrical transduction channels at the tips of the stereocilia from the middle and short rows, while bundle cohesion is maintained owing to the presence of horizontal top connectors. Here, we used a quantitative noncontact atomic force microscopy method to investigate stereocilia bundle stiffness and damping, when stimulated at acoustic frequencies and nanometer distances from the bundle. Stereocilia bundle mechanics were determined in stereocilin-deficient mice lacking top connectors and with detached tectorial membrane (Strc−/−/Tecta−/− double knockout) and heterozygous littermate controls (Strc+/−/Tecta−/−). A substantial decrease in bundle stiffness and damping by ~60 and ~74% on postnatal days P13 to P15 was observed when top connectors were absent. Additionally, we followed bundle mechanics during OHC top connectors development between P9 and P15 and quantified the observed increase in OHC bundle stiffness and damping in Strc+/−/Tecta−/− mice while no significant change was detected in Strc−/−/Tecta−/− animals.

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Mandelker, Diana, Sami S. Amr, Trevor Pugh, Sivakumar Gowrisankar, Rimma Shakhbatyan, Elizabeth Duffy, Mark Bowser et al. "Comprehensive Diagnostic Testing for Stereocilin". Journal of Molecular Diagnostics 16, n. 6 (novembre 2014): 639–47. http://dx.doi.org/10.1016/j.jmoldx.2014.06.003.

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Verpy, Elisabeth, Michel Leibovici, Nicolas Michalski, Richard J. Goodyear, Carine Houdon, Dominique Weil, Guy P. Richardson e Christine Petit. "Stereocilin connects outer hair cell stereocilia to one another and to the tectorial membrane". Journal of Comparative Neurology 519, n. 2 (16 dicembre 2010): 194–210. http://dx.doi.org/10.1002/cne.22509.

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Han, Woongsu, Jeong-Oh Shin, Ji-Hyun Ma, Hyehyun Min, Jinsei Jung, Jinu Lee, Un-Kyung Kim et al. "Distinct roles of stereociliary links in the nonlinear sound processing and noise resistance of cochlear outer hair cells". Proceedings of the National Academy of Sciences 117, n. 20 (1 maggio 2020): 11109–17. http://dx.doi.org/10.1073/pnas.1920229117.

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Outer hair cells (OHCs) play an essential role in hearing by acting as a nonlinear amplifier which helps the cochlea detect sounds with high sensitivity and accuracy. This nonlinear sound processing generates distortion products, which can be measured as distortion-product otoacoustic emissions (DPOAEs). The OHC stereocilia that respond to sound vibrations are connected by three kinds of extracellular links: tip links that connect the taller stereocilia to shorter ones and convey force to the mechanoelectrical transduction channels, tectorial membrane-attachment crowns (TM-ACs) that connect the tallest stereocilia to one another and to the overlying TM, and horizontal top connectors (HTCs) that link adjacent stereocilia. While the tip links have been extensively studied, the roles that the other two types of links play in hearing are much less clear, largely because of a lack of suitable animal models. Here, while analyzing genetic combinations of tubby mice, we encountered models missing both HTCs and TM-ACs or HTCs alone. We found that the tubby mutation causes loss of both HTCs and TM-ACs due to a mislocalization of stereocilin, which results in OHC dysfunction leading to severe hearing loss. Intriguingly, the addition of the modifier allele modifier of tubby hearing 1 in tubby mice selectively rescues the TM-ACs but not the HTCs. Hearing is significantly rescued in these mice with robust DPOAE production, indicating an essential role of the TM-ACs but not the HTCs in normal OHC function. In contrast, the HTCs are required for the resistance of hearing to damage caused by noise stress.

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Verpy, Elisabeth, Dominique Weil, Michel Leibovici, Richard J. Goodyear, Ghislaine Hamard, Carine Houdon, Gaelle M. Lefèvre et al. "Stereocilin-deficient mice reveal the origin of cochlear waveform distortions". Nature 456, n. 7219 (8 ottobre 2008): 255–58. http://dx.doi.org/10.1038/nature07380.

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Frykholm, Carina, Joakim Klar, Tatjana Tomanovic, Adam Ameur e Niklas Dahl. "Stereocilin gene variants associated with episodic vertigo: expansion of the DFNB16 phenotype". European Journal of Human Genetics 26, n. 12 (24 settembre 2018): 1871–74. http://dx.doi.org/10.1038/s41431-018-0256-6.

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Pacentine, Itallia, Paroma Chatterjee e Peter G. Barr-Gillespie. "Stereocilia Rootlets: Actin-Based Structures That Are Essential for Structural Stability of the Hair Bundle". International Journal of Molecular Sciences 21, n. 1 (3 gennaio 2020): 324. http://dx.doi.org/10.3390/ijms21010324.

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Sensory hair cells of the inner ear rely on the hair bundle, a cluster of actin-filled stereocilia, to transduce auditory and vestibular stimuli into electrical impulses. Because they are long and thin projections, stereocilia are most prone to damage at the point where they insert into the hair cell’s soma. Moreover, this is the site of stereocilia pivoting, the mechanical movement that induces transduction, which additionally weakens this area mechanically. To bolster this fragile area, hair cells construct a dense core called the rootlet at the base of each stereocilium, which extends down into the actin meshwork of the cuticular plate and firmly anchors the stereocilium. Rootlets are constructed with tightly packed actin filaments that extend from stereocilia actin filaments which are wrapped with TRIOBP; in addition, many other proteins contribute to the rootlet and its associated structures. Rootlets allow stereocilia to sustain innumerable deflections over their lifetimes and exemplify the unique manner in which sensory hair cells exploit actin and its associated proteins to carry out the function of mechanotransduction.

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Sathyanarayana, Bangalore K., Yoonsoo Hahn, Manish S. Patankar, Ira Pastan e Byungkook Lee. "Mesothelin, Stereocilin, and Otoancorin are predicted to have superhelical structures with ARM-type repeats". BMC Structural Biology 9, n. 1 (2009): 1. http://dx.doi.org/10.1186/1472-6807-9-1.

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Avenarius, Matthew R., Jocelyn F. Krey, Rachel A. Dumont, Clive P. Morgan, Connor B. Benson, Sarath Vijayakumar, Christopher L. Cunningham et al. "Heterodimeric capping protein is required for stereocilia length and width regulation". Journal of Cell Biology 216, n. 11 (12 settembre 2017): 3861–81. http://dx.doi.org/10.1083/jcb.201704171.

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Control of the dimensions of actin-rich processes like filopodia, lamellipodia, microvilli, and stereocilia requires the coordinated activity of many proteins. Each of these actin structures relies on heterodimeric capping protein (CAPZ), which blocks actin polymerization at barbed ends. Because dimension control of the inner ear’s stereocilia is particularly precise, we studied the CAPZB subunit in hair cells. CAPZB, present at ∼100 copies per stereocilium, concentrated at stereocilia tips as hair cell development progressed, similar to the CAPZB-interacting protein TWF2. We deleted Capzb specifically in hair cells using Atoh1-Cre, which eliminated auditory and vestibular function. Capzb-null stereocilia initially developed normally but later shortened and disappeared; surprisingly, stereocilia width decreased concomitantly with length. CAPZB2 expressed by in utero electroporation prevented normal elongation of vestibular stereocilia and irregularly widened them. Together, these results suggest that capping protein participates in stereocilia widening by preventing newly elongating actin filaments from depolymerizing.

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Tesi sul tema "Stereocilin"

Iranfar, Sepideh. "AAV-mediated gene therapy restores hearing and central auditory processing in a mouse model of human DFNB16 Deafness". Electronic Thesis or Diss., Sorbonne université, 2024. https://accesdistant.sorbonne-universite.fr/login?url=https://theses-intra.sorbonne-universite.fr/2024SORUS127.pdf.

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La déficience auditive constitue un handicap fonctionnel majeur, affectant plus d'un demi-milliard de personnes dans le monde. Malgré sa prévalence élevée, aucun traitement curatif n'existe actuellement. Mon projet de thèse est translationnel et vise à établir la preuve de concept selon laquelle la thérapie génique virale peut restaurer l'audition dans le modèle préclinique de surdité DNFB16. La surdité DFNB16 est la deuxième cause de déficience auditive congénitale d'origine génétique. Elle est causée par des mutations du gène codant pour la stéréociline (STRC) et se caractérise par une surdité légère à modérée. La protéine STRC est principalement exprimée dans les cellules ciliées externes (CCE) de l'oreille interne, l'un des deux types de cellules sensorielles de la cochlée, responsables de l'amplification et la discrimination fréquentielle du signal sonore. La protéine STRC est cruciale pour le maintien de la morphologie des stéréocils des CCE. Les mutations du gène STRC sont responsables d'un dysfonctionnement des CCE conduisant à l'abolition de l'amplification cochléaire et donc à une augmentation des seuils auditifs. A ce jour, il n'existe aucun traitement curatif pour la surdité DFNB16.L'objectif principal de mon projet était de développer une thérapie génique basée sur les virus adéno-associés (AAV) pour remplacer le gène mutant par une copie fonctionnelle dans un modèle murin DFNB16. Compte tenu de la grande taille de la séquence codante du gène Strc, dépassant la capacité d'empaquetage de l'AAV, j'ai utilisé une stratégie hybride de double vecteur pour charger l'ADNc de la Strc dans les capsides de l'AAV. Sachant que les CCE sont intrinsèquement difficiles à transduire par les vecteurs AAV, j'ai tout d'abord effectué une analyse comparative du tropisme cellulaire de différents sérotypes d'AAV après administration dans l'oreille interne afin d'identifier le la capside la plus efficace pour cibler les CCE. Ensuite, j'ai utilisé le sérotype AAV le plus performant pour construire le vecteur thérapeutique qui a été administré dans les cochlées des souris DFNB16.Les résultats montrent que la thérapie génique a rétabli une expression robuste de la protéine STRC et ciblée dans les touffes ciliaires des CCE chez les souris traitées. Cette expression a entraîné la restauration de la morphostructure des touffes ciliaires et de l'amplification cochléaire, permettant une récupération stable et durable des seuils auditifs, similaires à ceux de souris sauvages. Par ailleurs, les mesures psychométriques de la perception des fréquences à l'aide d'une tâche de Go/NoGO ont montré que la discrimination fréquentielle du signal sonore chez les souris DFNB16 traitées étaient comparables à celles des souris sauvages. Ces résultats soulignent l'efficacité de la thérapie génique sur la restauration de la perception sonore dans un modèle préclinique de surdité DFNB16. Cette découverte jette les bases d'une thérapie génique translationnelle efficace pour les patients atteints de DFNB16
Hearing impairment stands as a significant contributor to disability, affecting over half a billion individuals throughout their lifespans. Despite its pervasive prevalence, no curative treatment currently exists. My Ph.D. project is translational, aiming to establish the proof of concept that viral gene therapy can restore hearing in a preclinical model for DFNB16 deafness. DFNB16, considered the second most common cause of hearing impairment, is caused by mutations in the stereocilin (STRC) gene and is characterized by mild-to-moderate deafness. The stereocilin (STRC) protein is predominantly expressed in outer hair cells (OHCs), one of the two types of cochlear sensory hair cells, responsible for sound amplification. STRC protein is crucial for the cohesion and maintenance of OHC bundles. Mutations in STRC result in defective OHCs, leading to abolished cochlear amplification and subsequent reduction in hearing sensitivity. As of now, there exists no cure for DFNB16.My main objective was to develop an adeno-associated virus (AAV)-based gene therapy to replace the mutant gene with its correct copy in a DFNB16 mouse model. Given the large size of the Strc coding sequence, exceeding AAV packaging capacity, I employed a hybrid dual-vector strategy to load Strc cDNA into AAV capsids. Since OHCs are inherently difficult to transduce with AAV vectors, we firstly conducted a comparative analysis of AAV cellular tropism within the inner ear to identify the most efficient AAV serotype for targeting OHCs. Secondly, I used the best performing AAV serotype to construct the therapeutic vector, which was administered into the cochleas of DFNB16 mice.Following the gene therapy, we found a robust restoration of STRC protein expression and its appropriate targeting at the tips of OHC stereocilia in treated mice. This process results in the restoration of the normal morphostructure of OHC bundles and cochlear amplification, ensuring stable and long-lasting restoration of hearing in the treated mice, similar to those of the wild-type mice. Notably, psychometric measurements of frequency perception using a Go/NoGo task demonstrated that frequency discrimination exhibited by the treated Strc-/- mice was comparable to those of wild-type mice, underscoring the efficacy of gene therapy in recovering essential features of natural sound perception associated with DFNB16. This finding lays the foundation for effective translational gene therapy for DFNB16 patients and facilitates the development of preclinical gene therapy studies for mouse models of human deafness

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Gomez, Salvador Gustavo. "Protein Phosphatase 1 Concentrates at the Base of Sensory Hair Cell Stereocilia, Where it May Function in Stereocilia Cytoskeletal Structure". Ohio University Art and Sciences Honors Theses / OhioLINK, 2019. http://rave.ohiolink.edu/etdc/view?acc_num=ouashonors1556276688823712.

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Kitajiri, Shinichiro. "Radixin deficiency causes deafness associated with progressive degeneration of cochlear stereocilia". Kyoto University, 2005. http://hdl.handle.net/2433/144706.

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Peng, Anthony Wei. "A hair bundle proteomics approach to discovering actin regulatory proteins in inner ear stereocilia". Thesis, Massachusetts Institute of Technology, 2009. http://hdl.handle.net/1721.1/54588.

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Thesis (Ph. D.)--Harvard-MIT Division of Health Sciences and Technology, 2009.
Cataloged from PDF version of thesis.
Includes bibliographical references (p. 137-154).
Because there is little knowledge in the areas of stereocilia development, maintenance, and function in the hearing system, I decided to pursue a proteomics-based approach to discover proteins that play a role in stereocilia function. I employed a modified "twist-off" technique to isolate hair bundle proteins, and I developed a method to purify proteins and to process them for analysis using multi-dimensional protein identification technology (MudPIT). The MudPIT analysis yielded a substantial list of proteins. I verified the presence of 21 out of 34 (62%) existing proteins known to be present in stereocilia. This provided strong evidence that my proteomics approach was efficient in identifying hair bundle proteins. Next, I selected three proteins and localized them to murine cochlear stereocilia. StarD10, a putative phospholipid binding protein, was detectable along the shaft of stereocilia. Nebulin, a putative F-actin regulator, was located toward the base of stereocilia. Finally, twinfilin 2, a putative modulator of actin polymerization, was found at the tips of stereocilia. In order to determine the function of twinfilin 2, I localized the protein predominately to the tips of shorter stereocilia where it is up-regulated during the final phase of elongation. When overexpressed, I found that twinfilin 2 causes a shortening of microvilli in LLC-PK1/CL4 cells and in native cochlear stereocilia. The main result of this thesis was determining the sub-cellular localization of three interesting proteins and functionally characterizing one protein. My thesis also confirmed the proteomics screen I developed as an efficient method for identifying proteins in stereocilia.
by Anthony Wei Peng.
Ph.D.

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Shih-Wei, Chou. "FASCIN 2B IS A COMPONENT OF ZEBRAFISH STEREOCILIA AND A REGULATOR OF THEIR DIMENSIONS". Case Western Reserve University School of Graduate Studies / OhioLINK, 2015. http://rave.ohiolink.edu/etdc/view?acc_num=case1422016622.

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Hu, Jiaqi. "TARGETING MECHANOTRANSDUCTION-RELATED GENES OF THE HAIR CELLUSING TALEN AND CRISPR/CAS TECHNOLOGY". Case Western Reserve University School of Graduate Studies / OhioLINK, 2015. http://rave.ohiolink.edu/etdc/view?acc_num=case1417780489.

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Hwang, Philsang. "An In Vivo Study of the Function and Dynamics of Stereociliary Proteins". Case Western Reserve University School of Graduate Studies / OhioLINK, 2015. http://rave.ohiolink.edu/etdc/view?acc_num=case1418833642.

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Kulkarni, Prateek. "Interaction of MYO6 and CLIC5: An Interdependent Relation in the Hair Bundle Maintenance". Ohio University / OhioLINK, 2018. http://rave.ohiolink.edu/etdc/view?acc_num=ohiou1533730672111802.

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Waddell, Benjamin B. "CLIC5 maintains lifelong structural integrity of sensory stereocilia by promoting Radixin phosphorylation in hair cells of the inner ear". Ohio University Honors Tutorial College / OhioLINK, 2016. http://rave.ohiolink.edu/etdc/view?acc_num=ouhonors1461332124.

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Hadi, Shadan. "MYOSIN-XVA IS KEY MOLECULE IN ESTABLISHING THE ARCHITECTURE OF MECHANOSENSORY STEREOCILIA BUNDLES OF THE INNER EAR HAIR CELLS". UKnowledge, 2018. https://uknowledge.uky.edu/medsci_etds/9.

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Abstract (sommario):

Development of hair cell stereocilia bundles involves three stages: elongation, thickening, and supernumerary stereocilia retraction. Although Myo-XVa is known to be essential for stereocilia elongation, its role in retraction/thickening remains unknown. We quantified stereocilia numbers/diameters in shaker-2 mice (Myo15sh2) that have deficiencies in “long” and “short” isoforms of myosin-XVa, and in mice lacking only the “long” myosin-XVa isoform (Myo15ΔN). Our data showed that myosin-XVa is largely not involved in the developmental retraction of supernumerary stereocilia. In normal development, the diameters of the first (tallest)/second row stereocilia within a bundle are equal and grow simultaneously. The diameter of the third row stereocilia increases together with that of taller stereocilia until P1-2 and then either decreases almost two-fold in inner hair cells (IHCs) or stays the same in outer hair cells (OHCs), resulting in a prominent diameter gradation in IHCs and less prominent in OHCs. Sh2 mutation abolishes this gradation in IHCs/OHCs. Stereocilia of all rows grow in diameters nearly equally in Myo15sh2/sh2 IHCs and OHCs. Conversely, ΔN mutation does not affect normal stereocilia diameter gradation until ~P8. Therefore, myosin-XVa “short” isoform is essential for developmental thinning of third row stereocilia, which causes diameter gradation within a hair bundle.

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Libri sul tema "Stereocilin"

Canlon, Barbara. The effect of acoustic trauma on the tectorial membrane, stereocilia, and hearing sensitivity: Possible mechanisms underlying damage, recovery, and protection. Stockholm, Sweden: Distributed by Almqvist & Wiksell Periodical Co., 1988.

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Capitoli di libri sul tema "Stereocilin"

Kita, Tomoko, Tatsuya Katsuno e Shin-ichiro Kitajiri. "Stereocilia". In Regenerative Medicine for the Inner Ear, 31–38. Tokyo: Springer Japan, 2014. http://dx.doi.org/10.1007/978-4-431-54862-1_4.

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Watson, Glen M., e Patricia Mire. "Stereocilia Based Mechanoreceptors of Sea Anemones". In Cell and Molecular Biology of the Ear, 19–39. Boston, MA: Springer US, 2000. http://dx.doi.org/10.1007/978-1-4615-4223-0_2.

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Peng, Anthony W., e Anthony J. Ricci. "Glass Probe Stimulation of Hair Cell Stereocilia". In Methods in Molecular Biology, 487–500. New York, NY: Springer New York, 2016. http://dx.doi.org/10.1007/978-1-4939-3615-1_27.

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Engstrom, B., E. Borg e B. Canlon. "Morphology of Stereocilia on Cochlear Hair Cells after Noise Exposure". In Basic and Applied Aspects of Noise-Induced Hearing Loss, 1–9. Boston, MA: Springer US, 1986. http://dx.doi.org/10.1007/978-1-4684-5176-4_1.

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Saunders, James C., Barbara Canlon e Ake Flock. "Mechanical Changes in Stereocilia Following Overstimulation: Observations and Possible Mechanisms". In Basic and Applied Aspects of Noise-Induced Hearing Loss, 11–29. Boston, MA: Springer US, 1986. http://dx.doi.org/10.1007/978-1-4684-5176-4_2.

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Satarić, Miljko, e Tomas Nemeš. "Calcium Signaling Along Actin Filaments in Stereocilia Controls Hair-Bundle Motility". In Nonlinear Dynamics of Nanobiophysics, 307–44. Singapore: Springer Nature Singapore, 2022. http://dx.doi.org/10.1007/978-981-19-5323-1_11.

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Richardson, G. P., I. J. Russell, R. Wasserkort e M. Hans. "Aminoglycoside Antibiotics and Lectins Cause Irreversible Increases in the Stiffness of Cochlear Hair-Cell Stereocilia". In Cochlear Mechanisms: Structure, Function, and Models, 57–65. Boston, MA: Springer US, 1989. http://dx.doi.org/10.1007/978-1-4684-5640-0_7.

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Vélez-Ortega, A. Catalina, e Gregory I. Frolenkov. "Visualization of Live Cochlear Stereocilia at a Nanoscale Resolution Using Hopping Probe Ion Conductance Microscopy". In Methods in Molecular Biology, 203–21. New York, NY: Springer New York, 2016. http://dx.doi.org/10.1007/978-1-4939-3615-1_12.

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Pickles, J. O., S. D. Comis e M. P. Osborne. "The Morphology of Stereocilia and their Cross-Links in Relation to Noise Damage in the Guinea Pig". In Basic and Applied Aspects of Noise-Induced Hearing Loss, 31–41. Boston, MA: Springer US, 1986. http://dx.doi.org/10.1007/978-1-4684-5176-4_3.

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Horner, K. C., Y. Cazals e A. Guilhaume. "Round Window Cochlear Microphonic and Atrophy of Short and Middle Stereocilia on Outer Hair Cells in Hydropic Cochleas in Guinea Pigs". In Cochlear Mechanisms: Structure, Function, and Models, 207–15. Boston, MA: Springer US, 1989. http://dx.doi.org/10.1007/978-1-4684-5640-0_26.

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Atti di convegni sul tema "Stereocilin"

Smith, Sonya T., e Richard Chadwick. "Nanofluidics of Mammalian Hearing". In ASME 2011 International Mechanical Engineering Congress and Exposition. ASMEDC, 2011. http://dx.doi.org/10.1115/imece2011-64729.

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The inner hair cell stereocilia bundle performs the role of transducer in mammalian hearing. Acoustic stimuli deflect the hair bundle to open ion channels, resulting in cation influx and the subsequent release of a neurotransmitter at the base of the cell. Hypotheses for this transduction include fluid shear-driven motion between the tectorial membrane and the reticular lamina to deflect the bundle. It is presumed that ‘molecular gates’ sense tension in tip-links that connect adjacent stepped rows of stereocilia to open the channels. However, almost nothing is known about the endolymphatic flow in the micron-sized gap surrounding the bundle and the nanoscale sized gaps between individual stereocilia rows and between individual bundles. Here we show with nanometer resolution, how each row of stereocilia, their associated tip links and gates and the corresponding flow patterns move in response to acoustical input.

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HACKNEY, C. M., B. WEST e D. N. FURNESS. "THE COMPOSITION OF LINKAGES BETWEEN STEREOCILIA". In Proceedings of the International Symposium. WORLD SCIENTIFIC, 2000. http://dx.doi.org/10.1142/9789812793980_0043.

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Vandaele, Mathieu, Bryan S. Joyce e Pablo A. Tarazaga. "Design and Characterization of Piezo-Based Stereocilia". In ASME 2013 Conference on Smart Materials, Adaptive Structures and Intelligent Systems. American Society of Mechanical Engineers, 2013. http://dx.doi.org/10.1115/smasis2013-3189.

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The hair cells in the cochlea are responsible for transforming sound-induced vibration into electrical signals. Damage to these hair cells is among the most common forms of hearing loss in the developed world. Researchers have studied various artificial hair cell (AHC) designs for replacing these hair cells. One such method uses piezoelectric beams to mimic the hair cell’s mechanoelectrical transduction. A piezoelectric beam will produce an electric potential from an applied sound pressure. In the literature, the response of the cochlea to sound pressures is often described using tuning curves. Tuning curves plot the sound pressure level at a given frequency which produces a particular displacement, velocity, or neuron firing rate. The work presented here examines using piezoelectric AHC’s to mimic cochlear hair cells by creating tuning curves of constant tip velocity and voltage. A piezoceramic (PZT) beam and a piezo film (PVDF) bending sensor are examined. An output feedback controller based on PID control is developed to vary the sound pressure from a speaker to create tuning curves for the piezoelectric AHC’s. The tuning curves for the piezoelectric beams are compared to measurements obtained from the biological cochlea.

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Thumati, Balaje T., D. Subbaram Naidu e Larry Stout. "A Neuro-Fuzzy Model for Simulating Outer Hair Cell of Human Cochlea". In ASME 2005 International Mechanical Engineering Congress and Exposition. ASMEDC, 2005. http://dx.doi.org/10.1115/imece2005-80644.

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Understanding the functioning of the human auditory system has been of interest for decades and many mathematical models have been developed based on experimental results. Many of these models address the key components of the human auditory system: outer ear, middle ear, and inner ear, which consists of cochlea and organ of corti. In this paper, a novel approach for human auditory model is developed that is based on the concepts of fuzzy logic for simulating basilar membrane and stereocilia, and a feed-forward neural network for simulating outer hair cell of the inner ear. Frequency, intensity and the direction of stereocilia movement are the three inputs to the fuzzy logic portion of the model. The output of this block is the net force, which becomes the input to the neural network. The implementation and simulated results using MATLAB® are presented.

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Bathe, Mark, Claus Heussinger, Mireille Claessens, Andreas Bausch e Erwin Frey. "Cytoskeletal Bundle Mechanics". In ASME 2007 Summer Bioengineering Conference. American Society of Mechanical Engineers, 2007. http://dx.doi.org/10.1115/sbc2007-176170.

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Filamentous actin (F-actin) is a stiff biopolymer that is tightly crosslinked in vivo by actin-binding proteins (ABPs) to form stiff bundles that form major constituents of a multitude of slender cytoskeletal processes including stereocilia, filopodia, microvilli, neurosensory bristles, cytoskeletal stress fibers, and the acrosomal process of sperm cells (Fig. 1). The mechanical properties of these cytoskeletal processes play key roles in a broad range of cellular functions — the bending stiffness of stereocilia mediates the mechanochemical transduction of mechanical stimuli such as acoustic waves to detect sound, the critical buckling load of filopodia and acrosomal processes determines their ability to withstand compressive mechanical forces generated during cellular locomotion and fertilization, and the entropic stretching stiffness of cytoskeletal bundles mediates cytoskeletal mechanical resistance to cellular deformation. Thus, a detailed understanding of F-actin bundle mechanics is fundamental to gaining a mechanistic understanding of cytoskeletal function.

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GUETA, R., D. BARLAM, R. Z. SHNECK e I. ROUSSO. "THE ANISOTROPY OF THE TECTORIAL MEMBRANE GUIDES STEREOCILIA DEFLECTION". In Proceedings of the 10th International Workshop on the Mechanics of Hearing. WORLD SCIENTIFIC, 2009. http://dx.doi.org/10.1142/9789812833785_0040.

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LANGER, M. G., S. FINK, K. LÖFFLER, A. KOITSCHEV e H. P. ZENNER. "INVESTIGATION OF THE MECHANOELECTRICAL TRANSDUCTION AT SINGLE STEREOCILIA BY AFM". In Proceedings of the International Symposium. WORLD SCIENTIFIC, 2003. http://dx.doi.org/10.1142/9789812704931_0005.

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CHIARADIA, CAIO, MANUELA NOWOTNY e ANTHONY W. GUMMER. "DEFLECTION OF IHC STEREOCILIA IN RESPONSE TO SOMATIC OHC ELECTROMOTILITY". In Proceedings of the 10th International Workshop on the Mechanics of Hearing. WORLD SCIENTIFIC, 2009. http://dx.doi.org/10.1142/9789812833785_0044.

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Maftoon, Nima, Hamid Motallebzadeh, John J. Guinan e Sunil Puria. "Drive mechanisms to the inner and outer hair cell stereocilia". In TO THE EAR AND BACK AGAIN - ADVANCES IN AUDITORY BIOPHYSICS: Proceedings of the 13th Mechanics of Hearing Workshop. Author(s), 2018. http://dx.doi.org/10.1063/1.5038510.

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Altoè, Alessandro, e Ville Pulkki. "Deriving stereocilia displacement from the impedance of the organ of Corti". In MECHANICS OF HEARING: PROTEIN TO PERCEPTION: Proceedings of the 12th International Workshop on the Mechanics of Hearing. AIP Publishing LLC, 2015. http://dx.doi.org/10.1063/1.4939328.

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Rapporti di organizzazioni sul tema "Stereocilin"

Yazdidoust, Ladan. Defining Protein Interactions: Ankle Link Proteins of Stereocilia in Hair Cells. Portland State University Library, gennaio 2016. http://dx.doi.org/10.15760/honors.276.

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