scholarly journals A metabolomic and systems biology perspective on the brain of the Fragile X syndrome mouse model

2011 ◽  
Vol 21 (12) ◽  
pp. 2190-2202 ◽  
Author(s):  
L. Davidovic ◽  
V. Navratil ◽  
C. M. Bonaccorso ◽  
M. V. Catania ◽  
B. Bardoni ◽  
...  
Keyword(s):  
Systems Biology ◽  
Mouse Model ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
The Brain

scholarly journals Pioglitazone improves deficits of Fmr1-KO mouse model of Fragile X syndrome by interfering with excessive diacylglycerol signaling

2020 ◽  
Author(s):  
Andréa Geoffroy ◽  
Karima Habbas ◽  
Boglarka Zambo ◽  
Laetitia Schramm ◽  
Arnaud Duchon ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Rna Binding ◽  
Fragile X ◽  
Loss Of Function ◽  
Behavioral Alterations ◽  
Specific Proteins ◽  
The Brain

AbstractFragile X syndrome (FXS), the leading cause of familial intellectual disability, is an uncured disease caused by the absence or loss of function of the FMRP protein. FMRP is an RNA binding protein that controls the translation of specific proteins in neurons. A main target of FMRP in neurons is diacylglycerol kinase kappa (DGKk) and the loss of FMRP leads to a loss of DGK activity causing a diacylglycerol excess in the brain. Excessive diacylglycerol signaling could be a significant contributor to the pathomechanism of FXS. Here we tested the contribution of DAG-signaling in Fmr1-KO mouse model of FXS and we show that pioglitazone, a widely prescribed drug for type 2 diabetes, has ability to correct excessive DAG signaling in the brain and rescue behavioral alterations of the Fmr1-KO mouse. This study highlights the role of lipid signaling homeostasis in FXS and provides arguments to support the testing of pioglitazone for treatment of FXS.

scholarly journals Nanoparticle delivery of CRISPR into the brain rescues a mouse model of fragile X syndrome from exaggerated repetitive behaviours

2018 ◽  
Vol 2 (7) ◽  
pp. 497-507 ◽  
Author(s):  
Bumwhee Lee ◽  
Kunwoo Lee ◽  
Shree Panda ◽  
Rodrigo Gonzales-Rojas ◽  
Anthony Chong ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
Repetitive Behaviours ◽  
Nanoparticle Delivery ◽  
The Brain

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
Sign in / Sign up

Export Citation Format

Share Document