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Teses / dissertações sobre o tema "Stereocilin"

Autor: Grafiati
Publicado: 7 de setembro de 2024

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1

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 surdi
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2

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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3

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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4

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.<br>Cataloged from PDF version of thesis.<br>Includes bibliographical references (p. 137-154).<br>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 ide
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5

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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6

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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7

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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8

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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9

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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10

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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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 developmen
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11

Syam, Diana. "Immobilizing Mutation in an Unconventional Myosin15a Affects not only the Structure of Mechanosensory Stereocilia in the Inner Ear Hair Cells but also their Ionic Conductances." UKnowledge, 2014. http://uknowledge.uky.edu/medsci_etds/2.

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In the inner and outer hair cells (OHCs) of the inner ear, an unconventional myosin 15a localizes at the tips of mechanosensory stereocilia and plays an important role in forming and maintaining their normal structure. A missense mutation makes the motor domain of myosin 15a dysfunctional and is responsible for the congenital deafness DFNB3 in humans and deafness and vestibular defects in Shaker-2 (Sh2) mouse model. All hair cells of homozygous Shaker-2 mice (Myo15sh2/sh2) have abnormally short stereocilia, but, only stereocilia of Myo15sh2/sh2OHCs start to degenerate after the first few days
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12

Ricaud, Bruno. "Système de reconstruction d'environnement pour une aide au pilotage en environnement naturel." Thesis, Paris Sciences et Lettres (ComUE), 2016. http://www.theses.fr/2016PSLEM018/document.

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Le pilotage de véhicule blindé est rendu difficile par la faible visibilité offerte aux pilotes face aux environnements et aux situations complexes qu’ils doivent traverser.La protection des opérateurs de véhicules militaires et l’intégrité de ces véhicules sont des besoins primordiaux pour l’armée de terre.Afin de répondre à la problématique : sécuriser le pilotage des véhicules militaires avec comme périmètre la définition d’un système de perception d’environnement, nous avons procédé à l’étude au sens large de l’aide au pilotage dans le contexte militaire en environnement naturel et semi-st
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13

Xue, Jingbing. "Morphometry of Hair Cell Bundles and Otoconial Membranes in the Utricle of a Turtle, Trachemys scripta." Ohio : Ohio University, 2006. http://www.ohiolink.edu/etd/view.cgi?ohiou1155672444.

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14

Joshi, Yuvraj. "Mécanismes à l'origine de la surdité DFNA25." Thesis, Montpellier, 2020. http://www.theses.fr/2020MONTT041.

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Les problèmes auditifs affectent 16% des Européens, dont la moitié a plus de 60 ans, faisant de la presbyacousie, la surdité liée à l’âge, un véritable problème de santé publique. Chez l’homme, la mutation du gène SLC17A8, codant pour le transporteur vésiculaire du glutamate (VGLUT3) est à l’origine d’une surdité progressive prédominante sur les fréquences aigues (DFNA25), présentant ainsi les signes d’une presbyacousie précoce. Pour déterminer les mécanismes responsables de la surdité DFNA25, nous avons étudié le phénotype de la souris dont le gène SLC17A8 porte la mutation ponctuelle humaine
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15

Roy, Pallabi. "Stereocilia Morphogenesis and Maintenance is Dependent on the Dynamics of Actin Cytoskeletal Proteins." Thesis, 2019. http://hdl.handle.net/1805/18755.

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Indiana University-Purdue University Indianapolis (IUPUI)<br>Age-related hearing loss is an acute health problem affecting people worldwide, often arising due to defects in the proper functioning of sensory hair cells in the inner ear. The apical surface of sensory hair cells contains actin-based protrusions known as stereocilia, which detect sound and head movements. Since hair cells are not regenerated in mammals, it is important to maintain the functioning of stereocilia for the life of an organism to maintain hearing ability. The actin filaments within a stereocilium are extensively crossl
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16

(6481925), Pallabi Roy. "Stereocilia Morphogenesis and Maintenance is dependent on the Dynamics of Actin Cytoskeletal Proteins." Thesis, 2019.

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<p>Age-related hearing loss is an acute health problem affecting people worldwide, often arising due to defects in the proper functioning of sensory hair cells in the inner ear. The apical surface of sensory hair cells contains actin-based protrusions known as stereocilia, which detect sound and head movements. Since hair cells are not regenerated in mammals, it is important to maintain the functioning of stereocilia for the life of an organism to maintain hearing ability. The actin filaments within a stereocilium are extensively crosslinked by various actin crosslinking proteins, which are im
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17

McGrath, Jamis. "The role of ADF and cofilin in auditory sensory cell development." Thesis, 2020. http://hdl.handle.net/1805/24883.

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Indiana University-Purdue University Indianapolis (IUPUI)<br>Our ability to hear relies on sensory cells found in the inner ear that transduce sound into biological signals. Microvilli-like protrusions called stereocilia are bundled on the apical surfaces of these cells and allow them to respond to sound-evoked vibrations. The architecture of the stereocilia bundle is highly patterned to ensure normal hearing. Filaments of polymerized actin proteins are bundled in parallel into large cylindrical structures that define the dimensions of stereocilia. This network is then anchored to the cell by
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