Impaired GABA Neural Circuits Are Critical for Fragile X Syndrome.

Neural Plast

Department of Anesthesiology, Heze Municipal Hospital, Heze, 274031 Shandong, China.

Published: January 2019

Fragile X syndrome (FXS) is an inheritable neuropsychological disease caused by silence of the gene and the deficiency of Fragile X mental retardation protein (FMRP). Patients present neuronal alterations that lead to severe intellectual disability and altered sleep rhythms. However, the neural circuit mechanisms underlying FXS remain unclear. Previous studies have suggested that metabolic glutamate and gamma-aminobutyric acid (GABA) receptors/circuits are two counter-balanced factors involved in FXS pathophysiology. More and more studies demonstrated that attenuated GABAergic circuits in the absence of FMRP are critical for abnormal progression of FXS. Here, we reviewed the changes of GABA neural circuits that were attributed to intellectual-deficient FXS, from several aspects including deregulated GABA metabolism, decreased expressions of GABA receptor subunits, and impaired GABAergic neural circuits. Furthermore, the activities of GABA neural circuits are modulated by circadian rhythm of FMRP metabolism and reviewed the abnormal condition of FXS mice or patients.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6192167PMC
http://dx.doi.org/10.1155/2018/8423420DOI Listing

Publication Analysis

Top Keywords

neural circuits
16
gaba neural
12
fragile syndrome
8
fxs
6
neural
5
circuits
5
gaba
5
impaired gaba
4
circuits critical
4
critical fragile
4

Similar Publications

A significant proportion of patients who have recovered from COVID-19 suffer from persistent symptoms, referred to as "post-acute sequelae of SARS-CoV-2 infection (PASC)". Abnormal brain intrinsic activity has been observed in PASC patients, but the patterns of frequency-dependent intrinsic activity in the PASC and non-PASC (recovered COVID-19 patients without persistent symptoms) groups and their association with neuropsychiatric sequelae remain unclear in PASC. Twenty-nine PASC patients, 27 non-PASC subjects, and 31 healthy controls (HCs) were recruited.

View Article and Find Full Text PDF

Dendritic alterations precede age-related dysphagia and nucleus ambiguus motor neuron death.

J Physiol

January 2025

Department of Physiology & Biomedical Engineering, Mayo Clinic, Rochester, MN, USA.

Motor neurons (MNs) within the nucleus ambiguus innervate the skeletal muscles of the larynx, pharynx and oesophagus, which are essential for swallow. Disordered swallow (dysphagia) is a serious problem in elderly humans, increasing the risk of aspiration, a key contributor to mortality. Despite this importance, very little is known about the pathophysiology of ageing dysphagia and the relative importance of frank muscle weakness compared to timing/activation abnormalities.

View Article and Find Full Text PDF

Unlabelled: Motivated behaviors are regulated by distributed forebrain networks. Traditional approaches have often focused on individual brain regions and connections that do not capture the topographic organization of forebrain connectivity. We performed co-injections of anterograde and retrograde tract tracers in rats to provide novel high-spatial resolution evidence of topographic connections that elaborate a previously identified closed-loop forebrain circuit implicated in affective and motivational processes.

View Article and Find Full Text PDF

Optogenetics has transformed the study of neural circuit function, but limitations in its application to species with large brains, such as non-human primates (NHPs), remain. A major challenge in NHP optogenetics is delivering light to sufficiently large volumes of deep neural tissue with high spatiotemporal precision, without simultaneously affecting superficial tissue. To overcome these limitations, we recently developed and tested in NHP cortex, the Utah Optrode Array (UOA).

View Article and Find Full Text PDF

Unlabelled: Layer 6 corticothalamic (L6CT) neurons project to both cortex and thalamus, inducing multiple effects including the modulation of cortical and thalamic firing, and the emergence of high gamma oscillations in the cortical local field potential (LFP). We hypothesize that the high gamma oscillations driven by L6CT neuron activation are shaped by the dynamic engagement of intracortical and cortico-thalamo-cortical circuits. To test this, we optogenetically activated L6CT neurons in NTSR1-cre mice expressing channelrhodopsin-2 in L6CT neurons.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!