Altered neuronal and circuit excitability in fragile X syndrome

A Contractor, VA Klyachko, C Portera-Cailliau - Neuron, 2015 - cell.com
Fragile X syndrome (FXS) results from a genetic mutation in a single gene yet produces a
phenotypically complex disorder with a range of neurological and psychiatric problems …

The perineuronal 'safety'net? Perineuronal net abnormalities in neurological disorders

TH Wen, DK Binder, IM Ethell… - Frontiers in molecular …, 2018 - frontiersin.org
Perineuronal nets (PNN) are extracellular matrix (ECM) assemblies that preferentially
ensheath parvalbumin (PV) expressing interneurons. Converging evidence indicates that …

Chronic pharmacological mGlu5 inhibition corrects fragile X in adult mice

A Michalon, M Sidorov, TM Ballard, L Ozmen… - Neuron, 2012 - cell.com
Fragile X syndrome (FXS) is the most common form of inherited intellectual disability.
Previous studies have implicated mGlu5 in the pathogenesis of the disease, but a crucial …

[图书][B] What's wrong with my mouse?: behavioral phenotyping of transgenic and knockout mice

JN Crawley - 2007 - books.google.com
Dr. Jacqueline N. Crawley, author of the First and Second Editions of What's Wrong with My
Mouse? Behavioral Phenotyping of Transgenic and Knockout Mice, continues to field calls …

Prepulse inhibition in psychiatric disorders–apart from schizophrenia

S Kohl, K Heekeren, J Klosterkötter, J Kuhn - Journal of psychiatric …, 2013 - Elsevier
Prepulse inhibition (PPI) is a robust operational measure of sensorimotor gating. In
schizophrenic patients PPI is deficient. The aim of our review was to investigate the state of …

Imbalance of neocortical excitation and inhibition and altered UP states reflect network hyperexcitability in the mouse model of fragile X syndrome

JR Gibson, AF Bartley, SA Hays… - Journal of …, 2008 - journals.physiology.org
Despite the pronounced neurological deficits associated with mental retardation and autism,
it is unknown if altered neocortical circuit function occurs in these prevalent disorders. Here …

Mouse behavioral assays relevant to the symptoms of autism

JN Crawley - Brain pathology, 2007 - Wiley Online Library
While the cause of autism remains unknown, the high concordance between monozygotic
twins supports a strong genetic component. The importance of genetic factors in autism …

Modeling fragile X syndrome in the Fmr1 knockout mouse

TM Kazdoba, PT Leach, JL Silverman… - Intractable & rare …, 2014 - jstage.jst.go.jp
Summary Fragile X Syndrome (FXS) is a commonly inherited form of intellectual disability
and one of the leading genetic causes for autism spectrum disorder. Clinical symptoms of …

Realistic expectations of prepulse inhibition in translational models for schizophrenia research

NR Swerdlow, M Weber, Y Qu, GA Light, DL Braff - Psychopharmacology, 2008 - Springer
Introduction Under specific conditions, a weak lead stimulus, or “prepulse”, can inhibit the
startling effects of a subsequent intense abrupt stimulus. This startle-inhibiting effect of the …

Sensory processing in autism spectrum disorders and Fragile X syndrome—From the clinic to animal models

D Sinclair, B Oranje, KA Razak, SJ Siegel… - … & Biobehavioral Reviews, 2017 - Elsevier
Brains are constantly flooded with sensory information that needs to be filtered at the pre-
attentional level and integrated into endogenous activity in order to allow for detection of …