... to the native tissue. Hair cells Hearing loss Human inner ear organoids Otic Single-cell RNA-seq Ministerio de Ciencia, Innovación y Universidades PRE2021-097774 PID2020-117662GB-I00 Agencia Estatal de Investigación The inner ear is a sensory organ required...

.... , Jen , H. I. , Kang , H. , Klisch , T. J. , Zoghbi , H. Y. and Groves , A. K. ( 2015 ). Characterization of the transcriptome of nascent hair cells and identification of direct targets of the Atoh1 transcription factor . J. Neurosci. 35 , 5870 - 5883 . 10.1523/JNEUROSCI...

...Yoshitomo Ueda; Takashi Nakamura; Jing Nie; Alexander J. Solivais; John R. Hoffman; Becca J. Daye; Eri Hashino ABSTRACT The inner ear sensory epithelia contain mechanosensitive hair cells and supporting cells. Both cell types arise from SOX2-expressing prosensory cells, but the mechanisms...

...Amanda S. Janesick; Mirko Scheibinger; Nesrine Benkafadar; Sakin Kirti; Stefan Heller ABSTRACT The avian hearing organ is the basilar papilla that, in sharp contrast to the mammalian cochlea, can regenerate sensory hair cells and thereby recover from deafness within weeks. The mechanisms...

... F0 mice; these mice not only survived longer than regular Atoh1 / Sox10 knockout mice but also displayed their recognized cochlear phenotypes. Moreover, by using mosaic CRISPR-stop, we uncovered a previously unknown role of another lethal gene, Rbm24 , in the survival of cochlear outer hair cells...

...Maggie S. Matern; Beatrice Milon; Erika L. Lipford; Mark McMurray; Yoko Ogawa; Andrew Tkaczuk; Yang Song; Ran Elkon; Ronna Hertzano ABSTRACT Despite the known importance of the transcription factors ATOH1, POU4F3 and GFI1 in hair cell development and regeneration, their downstream transcriptional...

... . Recent advances in the field of stem cell biology and organoid culture systems allow the expansion and differentiation of tissue-specific progenitors and pluripotent stem cells in vitro into functional hair cells and otic-like neurons. We discuss how inner ear organoids have been developed and how...

..., or specific hair cells within them, is unclear. To address how the precise timing of Wnt signaling impacts patterning across the radial axis, mouse cochlear cultures were initiated at embryonic day 12.5 and subjected to pharmacological treatments at different stages. Early changes in major patterning genes...

...Israt Jahan; Ning Pan; Jennifer Kersigo; Bernd Fritzsch Atoh1 , a basic helix-loop-helix (bHLH) transcription factor (TF), is essential for the differentiation of hair cells (HCs), mechanotransducers that convert sound into auditory signals in the mammalian organ of Corti (OC). Previous work...

...Aida Costa; Luis Sanchez-Guardado; Stephanie Juniat; Jonathan E. Gale; Nicolas Daudet; Domingos Henrique ABSTRACT Mechanosensory hair cells (HCs) are the primary receptors of our senses of hearing and balance. Elucidation of the transcriptional networks regulating HC fate determination...

... in hair cell determination. These mechanisms depend respectively on two different ligands, jagged 1 (Jag1) and delta 1 (Dl1), that rely on a common signaling cascade initiated after Notch activation. In the chicken otocyst, expression of Jag1 and the Notch target Hey1 correlates well with lateral...

...Tomoko Tateya; Itaru Imayoshi; Ichiro Tateya; Kiyomi Hamaguchi; Hiroko Torii; Juichi Ito; Ryoichiro Kageyama Mechanosensory hair cells and supporting cells develop from common precursors located in the prosensory domain of the developing cochlear epithelium. Prosensory cell differentiation...

...Taha Adnan Jan; Renjie Chai; Zahra Nabi Sayyid; Renée van Amerongen; Anping Xia; Tian Wang; Saku Tapani Sinkkonen; Yi Arial Zeng; Jared Ruben Levin; Stefan Heller; Roel Nusse; Alan Gi-Lun Cheng Permanent hearing loss is caused by the irreversible damage of cochlear sensory hair cells and nonsensory...

.... In the inner ear, this pathway has been shown to regulate the size of the otic placode from which the cochlea will arise; however, direct activity of canonical Wnt signaling as well as its function during cochlear mechanosensory hair cell development had yet to be identified. Using TCF/Lef:H2B-GFP reporter...

...Stuart W. Webb; Nicolas Grillet; Leonardo R. Andrade; Wei Xiong; Lani Swarthout; Charley C. Della Santina; Bechara Kachar; Ulrich Müller Protocadherin 15 (PCDH15) is expressed in hair cells of the inner ear and in photoreceptors of the retina. Mutations in PCDH15 cause Usher Syndrome (deaf...

...Indra Wibowo; Filipe Pinto-Teixeira; Chie Satou; Shin-ichi Higashijima; Hernán López-Schier Bilateral symmetric tissues must interpret axial references to maintain their global architecture during growth or repair. The regeneration of hair cells in the zebrafish lateral line, for example, forms...

...Adèle Faucherre; Jean-Pierre Baudoin; Jesús Pujol-Martí; Hernán López-Schier The polarity of apical stereocilia endows hair cells with directional excitability, which in turn enables animals to determine the vectorial component of a sound. Neuromasts of the lateral line of aquatic vertebrates...

... with dilated cardiomyopathy, we studied eya4 expression during zebrafish development and characterized eya4 deficiency. eya4 morphant fish embryos had reduced numbers of hair cells in the otic vesicle and lateral line neuromasts with impaired sensory responses. Analyses of candidate genes that are known...

... the neurogenic epithelium of the developing inner ear into specialized mechanosensory receptors. By Cre-loxP fate mapping, we show that vestibular sensory hair cells derive from a previously neurogenic region of the inner ear. The related bHLH genes Ngn1 ( Neurog1 ) and Math1 ( Atoh1 ) are required, respectively...

...Bonny B. Millimaki; Elly M. Sweet; Mary S. Dhason; Bruce B. Riley Hair cells of the inner ear develop from an equivalence group marked by expression of the proneural gene Atoh1 . In mouse, Atoh1 is necessary for hair cell differentiation, but its role in specifying the equivalence group (proneural...