Cargando…

PKHD1L1 is a coat protein of hair-cell stereocilia and is required for normal hearing

The bundle of stereocilia on inner ear hair cells responds to subnanometer deflections produced by sound or head movement. Stereocilia are interconnected by a variety of links and also carry an electron-dense surface coat. The coat may contribute to stereocilia adhesion or protect from stereocilia f...

Descripción completa

Detalles Bibliográficos
Autores principales:	Wu, Xudong, Ivanchenko, Maryna V., Al Jandal, Hoor, Cicconet, Marcelo, Indzhykulian, Artur A., Corey, David P.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group UK 2019
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6707252/ https://www.ncbi.nlm.nih.gov/pubmed/31444330 http://dx.doi.org/10.1038/s41467-019-11712-w

Ejemplares similares

Serial scanning electron microscopy of anti-PKHD1L1 immuno-gold labeled mouse hair cell stereocilia bundles
por: Ivanchenko, Maryna V., et al.
Publicado: (2020)

PKHD1L1, A Gene Involved in the Stereociliary Coat, Causes Autosomal Recessive Nonsyndromic Hearing Loss
por: Redfield, Shelby E., et al.
Publicado: (2023)

Electron Microscopy Techniques for Investigating Structure and Composition of Hair-Cell Stereociliary Bundles
por: Ivanchenko, Maryna V., et al.
Publicado: (2021)

Mechanotransduction current is essential for stability of the transducing stereocilia in mammalian auditory hair cells
por: Vélez-Ortega, A Catalina, et al.
Publicado: (2017)

Loss of ARHGEF6 Causes Hair Cell Stereocilia Deficits and Hearing Loss in Mice
por: Zhu, Chengwen, et al.
Publicado: (2018)

The 133-kDa N-terminal domain enables myosin 15 to maintain mechanotransducing stereocilia and is essential for hearing
por: Fang, Qing, et al.
Publicado: (2015)

LKB1 Is Required for the Development and Maintenance of Stereocilia in Inner Ear Hair Cells in Mice
por: Men, Yuqin, et al.
Publicado: (2015)

Absence of plastin 1 causes abnormal maintenance of hair cell stereocilia and a moderate form of hearing loss in mice
por: Taylor, Ruth, et al.
Publicado: (2015)

Mini-PCDH15 gene therapy rescues hearing in a mouse model of Usher syndrome type 1F
por: Ivanchenko, Maryna V., et al.
Publicado: (2023)

Hearing Sensitivity of Primates: Recurrent and Episodic Positive Selection in Hair Cells and Stereocilia Protein-Coding Genes
por: Moreira, Andreia, et al.
Publicado: (2021)

The organization of actin filaments in the stereocilia of cochlear hair cells
Publicado: (1980)

Inner hair cell stereocilia are embedded in the tectorial membrane
por: Hakizimana, Pierre, et al.
Publicado: (2021)

Radixin modulates the function of outer hair cell stereocilia
por: Prasad, Sonal, et al.
Publicado: (2020)

Control of stereocilia length during development of hair bundles
por: Krey, Jocelyn F., et al.
Publicado: (2023)

Actin filaments, stereocilia, and hair cells of the bird cochlea. I. Length, number, width, and distribution of stereocilia of each hair cell are related to the position of the hair cell on the cochlea
Publicado: (1983)

Actin filaments, stereocilia, and hair cells of the bird cochlea. II. Packing of actin filaments in the stereocilia and in the cuticular plate and what happens to the organization when the stereocilia are bent
Publicado: (1983)

Pkhd1(cyli/cyli) mice have altered renal Pkhd1 mRNA processing and hormonally sensitive liver disease
por: Yang, Chaozhe, et al.
Publicado: (2023)

Sound-induced length changes in outer hair cell stereocilia
por: Hakizimana, Pierre, et al.
Publicado: (2012)

Polarized condensates confer row identity of hair cell stereocilia
por: Shi, Yingdong, et al.
Publicado: (2022)

Regeneration of Stereocilia of Hair Cells by Forced Atoh1 Expression in the Adult Mammalian Cochlea
por: Yang, Shi-Ming, et al.
Publicado: (2012)

Anion Exchanger 1b in Stereocilia Is Required for the Functioning of Mechanotransducer Channels in Lateral-Line Hair Cells of Zebrafish
por: Lin, Yuan-Hsiang, et al.
Publicado: (2015)

NEDD4-family E3 ligase dysfunction due to PKHD1/Pkhd1 defects suggests a mechanistic model for ARPKD pathobiology
por: Kaimori, Jun-ya, et al.
Publicado: (2017)

Clarin‐2 is essential for hearing by maintaining stereocilia integrity and function
por: Dunbar, Lucy A, et al.
Publicado: (2019)

Simulation of the Response of the Inner Hair Cell Stereocilia Bundle to an Acoustical Stimulus
por: Smith, Sonya T., et al.
Publicado: (2011)

Liquid-liquid phase separation in hair cell stereocilia development and maintenance
por: Li, Jianchao
Publicado: (2023)

MANF supports the inner hair cell synapse and the outer hair cell stereocilia bundle in the cochlea
por: Ikäheimo, Kuu, et al.
Publicado: (2021)

Challenging Disease Ontology by Instances of Atypical PKHD1 and PKD1 Genetics
por: de Fallois, Jonathan, et al.
Publicado: (2021)

GRXCR2 Regulates Taperin Localization Critical for Stereocilia Morphology and Hearing
por: Liu, Chang, et al.
Publicado: (2018)

OSBPL2 encodes a protein of inner and outer hair cell stereocilia and is mutated in autosomal dominant hearing loss (DFNA67)
por: Thoenes, Michaela, et al.
Publicado: (2015)

Multi-isotope imaging mass spectrometry (MIMS) reveals slow protein turnover in hair-cell stereocilia
por: Zhang, Duan-Sun, et al.
Publicado: (2012)

Preliminary biochemical characterization of the stereocilia and cuticular plate of hair cells of the chick cochlea
Publicado: (1989)

Gelsolin Plays a Role in the Actin Polymerization Complex of Hair Cell Stereocilia
por: Mburu, Philomena, et al.
Publicado: (2010)

Alternative Splice Forms Influence Functions of Whirlin in Mechanosensory Hair Cell Stereocilia
por: Ebrahim, Seham, et al.
Publicado: (2016)

Stereocilia Rootlets: Actin-Based Structures That Are Essential for Structural Stability of the Hair Bundle
por: Pacentine, Itallia, et al.
Publicado: (2020)

β-Actin and γ-Actin Are Each Dispensable for Auditory Hair Cell Development But Required for Stereocilia Maintenance
por: Perrin, Benjamin J., et al.
Publicado: (2010)

Gene Transfer with AAV9-PHP.B Rescues Hearing in a Mouse Model of Usher Syndrome 3A and Transduces Hair Cells in a Non-human Primate
por: György, Bence, et al.
Publicado: (2018)

FCHSD1 and FCHSD2 Are Expressed in Hair Cell Stereocilia and Cuticular Plate and Regulate Actin Polymerization In Vitro
por: Cao, Huiren, et al.
Publicado: (2013)

The integrity of cochlear hair cells is established and maintained through the localization of Dia1 at apical junctional complexes and stereocilia
por: Ninoyu, Yuzuru, et al.
Publicado: (2020)

Heterodimeric capping protein is required for stereocilia length and width regulation
por: Avenarius, Matthew R., et al.
Publicado: (2017)

Class III myosins shape the auditory hair bundles by limiting microvilli and stereocilia growth
por: Lelli, Andrea, et al.
Publicado: (2016)

Cannot write session to /tmp/vufind_sessions/sess_rh16hjlif3vod9pedfbpdbmt12