scholarly journals Potential Involvement of Impaired BKCa Channel Function in Sensory Defensiveness and Some Behavioral Disturbances Induced by Unfamiliar Environment in a Mouse Model of Fragile X Syndrome

2017 ◽  
Vol 43 (3) ◽  
pp. 492-502 ◽  
Author(s):  
Maria Isabel Carreno-Munoz ◽  
Fabienne Martins ◽  
Maria Carmen Medrano ◽  
Elisabetta Aloisi ◽  
Susanna Pietropaolo ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
Bkca Channel ◽  
Behavioral Disturbances ◽  
Channel Function ◽  
Sensory Defensiveness

scholarly journals Therapeutic effects of Chlorzoxazone, a BKCa channel agonist, in a mouse model of Fragile X syndrome

2020 ◽  
Author(s):  
Valerie Lemaire-Mayo ◽  
Marion Piquemal ◽  
Wim E. Crusio ◽  
Eric Louette ◽  
Susanna Pietropaolo
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Neuronal Excitability ◽  
Fragile X ◽  
Drug Repurposing ◽  
Bkca Channel ◽  
Therapeutic Effects ◽  
Pharmacological Treatments ◽  
Bkca Channels ◽  
Behavioral Abnormalities

ABSTRACTFragile X syndrome (FXS) is an X-linked developmental disorder characterized by several behavioral abnormalities, including hyperactivity, sensory hyper-responsiveness and cognitive deficits, as well as autistic symptoms, e.g., reduced social interaction. These behavioural alterations are recapitulated by the major animal model of FXS, i.e., the Fmr1-KO mouse, which has been extensively employed to identify therapeutic targets for FXS, though effective pharmacological treatments are still lacking. Here we focused on the therapeutic role of large-conductance Calcium-dependent potassium (BKCa) channels, playing a crucial role in neuronal excitability and neurotransmitter release. Reduced expression/functionality of these channels has been described in FXS patients and mice, so that molecules activating these channels have been proposed as promising treatments for this syndrome. Here we performed an extensive characterization of the therapeutic impact of a novel BKCa agonist on FXS-like symptoms in the Fmr1-KO mouse model, employing a drug repurposing setting. We evaluated the acute and chronic effects of chlorzoxazone, i.e., a classical drug used for non-developmental muscular pathologies, on the locomotor, social, cognitive and sensory-motor alterations of Fmr1-KO mice and compared them with other pharmacological treatments recently proposed for FXS that instead do not target BKCa channels. Our results clearly demonstrate for the first time the marked efficacy of chlorzoxazone in treating all the behavioral abnormalities of FXS mice, thus encouraging the preferential use of this molecule over others for clinical applications in the field of FXS, and potentially of other neurodevelopmental disorders.

scholarly journals Impaired Dendritic Expression and Plasticity of h-Channels in the fmr1−/y Mouse Model of Fragile X Syndrome

Cell Reports ◽  
2012 ◽  
Vol 1 (3) ◽  
pp. 225-233 ◽  
Author(s):  
Darrin H. Brager ◽  
Arvin R. Akhavan ◽  
Daniel Johnston
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
H Channels

Mouse model of fragile X syndrome: Behavioral and hormonal response to stressors.

2009 ◽  
Vol 123 (3) ◽  
pp. 677-686 ◽  
Author(s):  
Darci M. Nielsen ◽  
Jeffrey J. Evans ◽  
William J. Derber ◽  
Kenzie A. Johnston ◽  
Mark L. Laudenslager ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
Hormonal Response

scholarly journals The PDE10A Inhibitor TAK-063 Reverses Sound-Evoked EEG Abnormalities in a Mouse Model of Fragile X Syndrome

2021 ◽  
Author(s):  
Carrie R. Jonak ◽  
Manbir S. Sandhu ◽  
Samantha A. Assad ◽  
Jacqueline A. Barbosa ◽  
Mahindra Makhija ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
Eeg Abnormalities

Aberrant differentiation of glutamatergic cells in neocortex of mouse model for fragile X syndrome

2009 ◽  
Vol 33 (2) ◽  
pp. 250-259 ◽  
Author(s):  
Topi A. Tervonen ◽  
Verna Louhivuori ◽  
Xiaohong Sun ◽  
Marie-Estelle Hokkanen ◽  
Claudius F. Kratochwil ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Fragile X

Modulation of behavioral phenotypes by a muscarinic M1 antagonist in a mouse model of fragile X syndrome

2011 ◽  
Vol 217 (1) ◽  
pp. 143-151 ◽  
Author(s):  
Surabi Veeraragavan ◽  
Nghiem Bui ◽  
Jennie R. Perkins ◽  
Lisa A. Yuva-Paylor ◽  
Randall L. Carpenter ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
Behavioral Phenotypes
Sign in / Sign up

Export Citation Format

Share Document