Cargando…

CNS-dominant human FMRP isoform rescues seizures, fear, and sleep abnormalities in Fmr1-KO mice

Fragile X syndrome is a neurodevelopmental disorder caused by the absence of the mRNA-binding protein fragile X messenger ribonucleoprotein (FMRP). Because FMRP is a highly pleiotropic protein controlling the expression of hundreds of genes, viral vector–mediated gene replacement therapy is viewed a...

Descripción completa

Detalles Bibliográficos
Autores principales:	Wong, Hayes, Hooper, Alexander W.M., Kang, Hye Ri, Lee, Shiron J., Zhao, Jiayi, Sadhu, Chanchal, Rawat, Satinder, Gray, Steven J., Hampson, David R.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	American Society for Clinical Investigation 2023
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10393223/ https://www.ncbi.nlm.nih.gov/pubmed/37288657 http://dx.doi.org/10.1172/jci.insight.169650

Ejemplares similares

Gene therapy using human FMRP isoforms driven by the human FMR1 promoter rescues fragile X syndrome mouse deficits
por: Jiang, Yiru, et al.
Publicado: (2022)

The Use of “Retardation” in FRAXA, FMRP, FMR1 and Other Designations
por: Herring, Jonathan, et al.
Publicado: (2022)

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

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

Elevated FMR1-mRNA and lowered FMRP – A double-hit mechanism for psychiatric features in men with FMR1 premutations
por: Schneider, Andrea, et al.
Publicado: (2020)

Association between IQ and FMR1 protein (FMRP) across the spectrum of CGG repeat expansions
por: Kim, Kyoungmi, et al.
Publicado: (2019)

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)

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

The G-quartet containing FMRP binding site in FMR1 mRNA is a potent exonic splicing enhancer
por: Didiot, Marie-Cécile, et al.
Publicado: (2008)

Single-Nucleotide Mutations in FMR1 Reveal Novel Functions and Regulatory Mechanisms of the Fragile X Syndrome Protein FMRP
por: Suhl, Joshua A., et al.
Publicado: (2015)

Sexually Divergent Mortality and Partial Phenotypic Rescue After Gene Therapy in a Mouse Model of Dravet Syndrome
por: Niibori, Yosuke, et al.
Publicado: (2020)

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)

Comment on Herring et al. The Use of “Retardation” in FRAXA, FMRP, FMR1 and Other Designations. Cells 2022, 11, 1044
por: Bruford, Elspeth
Publicado: (2022)

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)

CRISPR to the Rescue: Advances in Gene Editing for the FMR1 Gene
por: Yrigollen, Carolyn M., et al.
Publicado: (2019)

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)

Novel fragile X syndrome 2D and 3D brain models based on human isogenic FMRP-KO iPSCs
por: Brighi, Carlo, et al.
Publicado: (2021)

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)

The feasibility and utility of hair follicle sampling to measure FMRP and FMR1 mRNA in children with or without fragile X syndrome: a pilot study
por: Jalnapurkar, Isha, et al.
Publicado: (2022)

Dietary rescue of adult behavioral deficits in the Fmr1 knockout mouse
por: Nolan, Suzanne O., et al.
Publicado: (2022)

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

The effect of an mGluR5 inhibitor on procedural memory and avoidance discrimination impairments in Fmr1 KO mice
por: Veloz, M F Vinueza, et al.
Publicado: (2012)

New Insights Into the Role of Ca(v)2 Protein Family in Calcium Flux Deregulation in Fmr1-KO Neurons
por: Castagnola, Sara, et al.
Publicado: (2018)

Cellular and synaptic phenotypes lead to disrupted information processing in Fmr1-KO mouse layer 4 barrel cortex
por: Domanski, Aleksander P. F., et al.
Publicado: (2019)

Adenosine A(2A) receptor inhibition reduces synaptic and cognitive hippocampal alterations in Fmr1 KO mice
por: Ferrante, Antonella, et al.
Publicado: (2021)

Mammalian FMRP S499 Is Phosphorylated by CK2 and Promotes Secondary Phosphorylation of FMRP
por: Bartley, Christopher M., et al.
Publicado: (2016)

Dose-Dependent Rescue of KO Amelogenin Enamel by Transgenes in Vivo
por: Bidlack, Felicitas B., et al.
Publicado: (2017)

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)

Increased 2-arachidonoyl-sn-glycerol levels normalize cortical responses to sound and improve behaviors in Fmr1 KO mice
por: Pirbhoy, Patricia S., 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)

Hyperacusis in the Adult Fmr1-KO Mouse Model of Fragile X Syndrome: The Therapeutic Relevance of Cochlear Alterations and BKCa Channels
por: Ferraguto, Celeste, et al.
Publicado: (2023)

Retinoic Acid Supplementation Rescues the Social Deficits in Fmr1 Knockout Mice
por: Yang, Liqin, et al.
Publicado: (2022)

Fmr1 Transcript Isoforms: Association with Polyribosomes; Regional and Developmental Expression in Mouse Brain
por: Brackett, David M., et al.
Publicado: (2013)

Fmr1-KO mice failure to detect object novelty associates with a post-test decrease of structural and synaptic plasticity upstream of the hippocampus
por: Borreca, Antonella, et al.
Publicado: (2023)

Cannot write session to /tmp/vufind_sessions/sess_7a4v3vgpognm93qembvan5k1a5