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 …

A framework for the investigation of rare genetic disorders in neuropsychiatry

SJ Sanders, M Sahin, J Hostyk, A Thurm… - Nature medicine, 2019 - nature.com
De novo and inherited rare genetic disorders (RGDs) are a major cause of human morbidity,
frequently involving neuropsychiatric symptoms. Recent advances in genomic technologies …

Genetic reduction of matrix metalloproteinase-9 promotes formation of perineuronal nets around parvalbumin-expressing interneurons and normalizes auditory cortex …

TH Wen, S Afroz, SM Reinhard, AR Palacios… - Cerebral …, 2018 - academic.oup.com
Abnormal sensory responses associated with Fragile X Syndrome (FXS) and autism
spectrum disorders include hypersensitivity and impaired habituation to repeated stimuli …

Translation-relevant EEG phenotypes in a mouse model of Fragile X Syndrome

JW Lovelace, IM Ethell, DK Binder, KA Razak - Neurobiology of disease, 2018 - Elsevier
Identification of comparable biomarkers in humans and validated animal models will
facilitate pre-clinical to clinical therapeutic pipelines to treat neurodevelopmental disorders …

Sensory processing phenotypes in fragile X syndrome

M Rais, DK Binder, KA Razak, IM Ethell - ASN neuro, 2018 - journals.sagepub.com
Fragile X syndrome (FXS) is a neurodevelopmental disorder that causes intellectual
disability. It is a leading known genetic cause of autism. In addition to cognitive, social, and …

Of men and mice: modeling the fragile X syndrome

R Dahlhaus - Frontiers in molecular neuroscience, 2018 - frontiersin.org
The Fragile X Syndrome (FXS) is one of the most common forms of inherited intellectual
disability in all human societies. Caused by the transcriptional silencing of a single gene, the …

Increased aperiodic gamma power in young boys with Fragile X Syndrome is associated with better language ability

CL Wilkinson, CA Nelson - Molecular autism, 2021 - Springer
Background The lack of robust and reliable clinical biomarkers in Fragile X Syndrome (FXS),
the most common inherited form of intellectual disability, has limited the successful …

Auditory EEG biomarkers in fragile X syndrome: clinical relevance

LE Ethridge, LA De Stefano, LM Schmitt… - Frontiers in integrative …, 2019 - frontiersin.org
Sensory hypersensitivities are common and distressing features of Fragile X Syndrome
(FXS). While there are many drug interventions that reduce behavioral deficits in Fmr1 mice …

Clinical applications of EEG power spectra aperiodic component analysis: A mini-review

SM Pani, L Saba, M Fraschini - Clinical Neurophysiology, 2022 - Elsevier
Objective The present mini-review summarizes recent clinical findings related to the analysis
of the aperiodic component of EEG (electroencephalographic) power spectra, making them …

[HTML][HTML] Human models are needed for studying human neurodevelopmental disorders

X Zhao, A Bhattacharyya - The American Journal of Human Genetics, 2018 - cell.com
The analysis of animal models of neurological disease has been instrumental in furthering
our understanding of neurodevelopment and brain diseases. However, animal models are …