Cargando…

Hyperacusis in the Adult Fmr1-KO Mouse Model of Fragile X Syndrome: The Therapeutic Relevance of Cochlear Alterations and BKCa Channels

Hyperacusis, i.e., an increased sensitivity to sounds, is described in several neurodevelopmental disorders (NDDs), including Fragile X Syndrome (FXS). The mechanisms underlying hyperacusis in FXS are still largely unknown and effective therapies are lacking. Big conductance calcium-activated potass...

Descripción completa

Detalles Bibliográficos
Autores principales:	Ferraguto, Celeste, Bouleau, Yohan, Peineau, Thibault, Dulon, Didier, Pietropaolo, Susanna
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2023
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10380266/ https://www.ncbi.nlm.nih.gov/pubmed/37511622 http://dx.doi.org/10.3390/ijms241411863

Ejemplares similares

Rescue of fragile X syndrome phenotypes in Fmr1 KO mice by a BKCa channel opener molecule
por: Hébert, Betty, et al.
Publicado: (2014)

Synaptic Release Potentiation at Aging Auditory Ribbon Synapses
por: Peineau, Thibault, et al.
Publicado: (2021)

Early Administration of the Phytocannabinoid Cannabidivarin Prevents the Neurobehavioral Abnormalities Associated with the Fmr1-KO Mouse Model of Fragile X Syndrome
por: Premoli, Marika, et al.
Publicado: (2023)

Developmental Acquisition of a Rapid Calcium-Regulated Vesicle Supply Allows Sustained High Rates of Exocytosis in Auditory Hair Cells
por: Levic, Snezana, et al.
Publicado: (2011)

The Impact of Mild Chronic Stress and Maternal Experience in the Fmr1 Mouse Model of Fragile X Syndrome
por: Subashi, Enejda, et al.
Publicado: (2023)

Comparative Behavioral Phenotypes of Fmr1 KO, Fxr2 Het, and Fmr1 KO/Fxr2 Het Mice
por: Saré, Rachel Michelle, et al.
Publicado: (2019)

A synaptic F-actin network controls otoferlin-dependent exocytosis in auditory inner hair cells
por: Vincent, Philippe FY, et al.
Publicado: (2015)

Reversal of Fragile X Phenotypes by Manipulation of AβPP/Aβ Levels in Fmr1(KO) Mice
por: Westmark, Cara J., et al.
Publicado: (2011)

Neurocognitive endophenotypes in CGG KI and Fmr1 KO mouse models of Fragile X-Associated disorders: an analysis of the state of the field
por: Hunsaker, Michael R.
Publicado: (2013)

Sub-synaptic, multiplexed analysis of proteins reveals Fragile X related protein 2 is mislocalized in Fmr1 KO synapses
por: Wang, Gordon X, et al.
Publicado: (2016)

Long-term behavioral effects of prenatal stress in the Fmr1-knock-out mouse model for fragile X syndrome
por: Petroni, Valeria, et al.
Publicado: (2022)

Efficient Delivery of FMR1 across the Blood Brain Barrier Using AAVphp Construct in Adult FMR1 KO Mice Suggests the Feasibility of Gene Therapy for Fragile X Syndrome
por: Chadman, Kathryn K., et al.
Publicado: (2023)

Ventral cochlear nucleus bushy cells encode hyperacusis in guinea pigs
por: Martel, David T., et al.
Publicado: (2020)

Fmr1 KO Mice as a Possible Model of Autistic Features
por: Bernardet, Maude, et al.
Publicado: (2006)

Homeostatic Intrinsic Plasticity Is Functionally Altered in Fmr1 KO Cortical Neurons
por: Bülow, Pernille, et al.
Publicado: (2019)

Revisiting the Large-Conductance Calcium-Activated Potassium (BKCa) Channels in the Pulmonary Circulation
por: Guntur, Divya, et al.
Publicado: (2021)

Hyperexcitability and Loss of Feedforward Inhibition Contribute to Aberrant Plasticity in the Fmr1KO Amygdala
por: Svalina, Matthew N., et al.
Publicado: (2021)

Early Neurobehavioral Characterization of the CD Mouse Model of Williams–Beuren Syndrome
por: Giannoccaro, Silvia, et al.
Publicado: (2023)

Reevaluation of FMR1 Hypermethylation Timing in Fragile X Syndrome
por: Mor-Shaked, Hagar, et al.
Publicado: (2018)

Autistic-like behavioral effects of prenatal stress in juvenile Fmr1 mice: the relevance of sex differences and gene–environment interactions
por: Petroni, Valeria, et al.
Publicado: (2022)

Effects of RMF on BKCa and Kv channels in basilar arterial smooth-muscle cells of SHR
por: Qian, Yan-Fei, et al.
Publicado: (2017)

A sound-driven cortical phase-locking change in the Fmr1 KO mouse requires Fmr1 deletion in a subpopulation of brainstem neurons
por: Holley, Andrew J., et al.
Publicado: (2022)

In Vivo Volume Dynamics of Dendritic Spines in the Neocortex of Wild-Type and Fmr1 KO Mice
por: Ishii, Kazuhiko, et al.
Publicado: (2018)

Development and validation of Hyperacusis Handicap Questionnaire in individuals with tinnitus associated with hyperacusis
por: Prabhu, Prashanth, et al.
Publicado: (2020)

Evaluation of FMR4, FMR5 and FMR6 Expression Levels as Non-Invasive Biomarkers for the Diagnosis of Fragile X-Associated Primary Ovarian Insufficiency (FXPOI)
por: Alvarez-Mora, Maria Isabel, et al.
Publicado: (2022)

High performing male with fragile X syndrome with an unmethylated FMR1 full mutation: The relevance of clinical and genetic correlations
por: Shieh, Meg, et al.
Publicado: (2023)

Hyperacusis Assessment Questionnaire—A New Tool Assessing Hyperacusis in Subjects with Tinnitus
por: Raj-Koziak, Danuta, et al.
Publicado: (2023)

Depressed nNOS expression during spine transition in the developing hippocampus of FMR1 KO mice
por: Xu, Qin, et al.
Publicado: (2012)

Impaired Adaptation and Laminar Processing of the Oddball Paradigm in the Primary Visual Cortex of Fmr1 KO Mouse
por: Pak, Alexandr, et al.
Publicado: (2021)

The short hyperacusis questionnaire: A tool for the identification and measurement of hyperacusis in the Italian tinnitus population
por: Tortorella, Federica, et al.
Publicado: (2017)

Spontaneous Coronary Artery Dissection in Females With the Fragile X FMR1 Premutation
por: McKenzie, Forrest J., et al.
Publicado: (2020)

Pirenperone relieves the symptoms of fragile X syndrome in Fmr1 knockout mice
por: Kim, Yujeong, et al.
Publicado: (2022)

Testing Fmr1(KO) Phenotypes in Response to GSK3 Inhibitors: SB216763 versus AFC03127
por: Westmark, Pamela R., et al.
Publicado: (2021)

Largely unaffected auditory and visual sensory processing phenotypes in the evoked potentials of Fmr1 KO2 mice
por: Kat, Renate, et al.
Publicado: (2022)

Evaluating the effect of R-Baclofen and LP-211 on autistic behavior of the BTBR and Fmr1-KO mouse models
por: Sharghi, Shirin, et al.
Publicado: (2023)

CNS-dominant human FMRP isoform rescues seizures, fear, and sleep abnormalities in Fmr1-KO mice
por: Wong, Hayes, et al.
Publicado: (2023)

Caveolin-1 Limits the Contribution of BKCa Channel to MCF-7 Breast Cancer Cell Proliferation and Invasion
por: Du, Cheng, et al.
Publicado: (2014)

Phenotypic Characteristics of Hyperacusis in Tinnitus
por: Schecklmann, Martin, et al.
Publicado: (2014)

Audiometric Characteristics of Hyperacusis Patients
por: Sheldrake, Jacqueline, et al.
Publicado: (2015)

Characteristics of Hyperacusis in the General Population
por: Paulin, Johan, et al.
Publicado: (2016)

Cannot write session to /tmp/vufind_sessions/sess_ijvjth9mpvn289p2b5oa7pil90