Abstract:
:Fragile X syndrome (FXS) is a neurodevelopmental disorder characterized by intellectual disability, sensory hypersensitivity, and high incidences of autism spectrum disorders and epilepsy. These phenotypes are suggestive of defects in neural circuit development and imbalances in excitatory glutamatergic and inhibitory GABAergic neurotransmission. While alterations in excitatory synapse function and plasticity are well-established in Fmr1 knockout (KO) mouse models of FXS, a number of recent electrophysiological and molecular studies now identify prominent defects in inhibitory GABAergic transmission in behaviorally relevant forebrain regions such as the amygdala, cortex, and hippocampus. In this review, we summarize evidence for GABAergic system dysfunction in FXS patients and Fmr1 KO mouse models alike. We then discuss some of the known developmental roles of GABAergic signaling, as well as the development and refinement of GABAergic synapses as a framework for understanding potential causes of mature circuit dysfunction. Finally, we highlight the GABAergic system as a relevant target for the treatment of FXS.
journal_name
Dev Neuroscijournal_title
Developmental neuroscienceauthors
Paluszkiewicz SM,Martin BS,Huntsman MMdoi
10.1159/000329420subject
Has Abstractpub_date
2011-01-01 00:00:00pages
349-64issue
5eissn
0378-5866issn
1421-9859pii
000329420journal_volume
33pub_type
杂志文章,评审abstract::Chick embryo retina neurons in culture extend neurites and form synapses. Seven different culture substrates were compared to determine which culture surface would produce the best neuronal survival and neurite extension. The order of survival of retina neurons during 7 days of culture is salt-precipitated collagen gr...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112705
更新日期:1982-01-01 00:00:00
abstract::Dissociated cells from chick embryo encephalon of 36 h of incubation have been cultivated on fibroblast, meningeal cell, or glial layers. Within the first 24 h of cultivation, cells adhered to the preformed layers more firmly than to plastic or collagen surfaces. After 48 h of cultivation, the brain cells cultured on ...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112568
更新日期:1978-01-01 00:00:00
abstract:BACKGROUND:Therapeutic hypothermia provides incomplete neuroprotection for neonatal hypoxic-ischemic encephalopathy (HIE). We examined whether hemodynamic goals that support autoregulation are associated with decreased brain injury and whether these relationships are affected by birth asphyxia or vary by anatomic regio...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000452833
更新日期:2017-01-01 00:00:00
abstract::Uncertainty reigns over whether or not glutamate uptake in astrocytes leads to strong stimulation of glucose utilization, measured as accumulation of radioactive deoxyglucose-6-phosphate. This is an important issue, not only because glutamate is the major excitatory transmitter, but also because it has been postulated...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000017329
更新日期:1998-01-01 00:00:00
abstract::In the era of therapeutic hypothermia, reliable preclinical studies are integral to successfully identify neuroprotective strategies to further improve outcomes of encephalopathy at term. We reviewed preclinical neuroprotection studies reported between January 2014 and June 2016 to assess the use of effective temperat...
journal_title:Developmental neuroscience
pub_type: 杂志文章,评审
doi:10.1159/000452859
更新日期:2017-01-01 00:00:00
abstract::Dissociated cells of cerebral hemispheres from 8-day-old chick embryos were cultivated for 8 days in polylysine-coated Petri dishes. Changes of DNA, RNA, total proteins, putrescine, spermidine, spermine, free amino acids and enzymes involved in polyamine and GABA metabolism were studied throughout neuronal development...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112755
更新日期:1981-01-01 00:00:00
abstract::Protein synthetic activity of anterior hypothalamic and neocortical brain slices from androgenized neonatal female rats was studied in vitro in an attempt to clarify the mechanism of action of testosterone on the developing hypothalamus. The short-term effects of testosterone propionate injections of the animals on th...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112449
更新日期:1979-01-01 00:00:00
abstract::Central nervous system (CNS) stem cells require epidermal growth factor (EGF) to survive and express glial-specific proteins (GSPs) under its influence. EGF and its receptor (EGF-R) therefore are involved in gliogenesis. We hypothesize that EGF selectively modulates GSP expression in pluripotential CNS cells and this ...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000048699
更新日期:2001-01-01 00:00:00
abstract::Neurotoxicological studies have indicated that L-glutamate exhibits more pronounced effects on the preoptic area (POA) neurons of male rats than on those of females in the neonatal period. However, no information has previously been available as to whether or not such sexual dimorphism also exists for the effects of g...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000048726
更新日期:2001-01-01 00:00:00
abstract::The distal segments of the crush-injured and permanently transected sciatic nerve provide models to study Schwann cell activity in the presence and absence of Schwann cell-axonal contact, respectively. We examined the quantity and quality of transcript coding for the myelin-associated glycoprotein (MAG) over a 3-week ...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000111832
更新日期:1990-01-01 00:00:00
abstract::The postnatal developmental expression and the distribution of the glutamate transporters (GLAST, GLT-1 and EAAC1) were analyzed in rat vestibular nuclei (VN), at birth and during the following 4 weeks. Analyses were performed using reverse transcriptase-polymerase chain reaction and immunoblotting of GLAST, GLT-1 and...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000073510
更新日期:2003-09-01 00:00:00
abstract::Using Western blot and immunocytochemistry to detect protein, and in situ hybridization and Northern blot to detect RNA, we studied the expression of Na,K-ATPase subunit isoforms in cultured astrocytes and oligodendrocytes in vitro, and in glial cells in the brain. We found that astrocytes in culture express alpha 1 a...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000111422
更新日期:1996-01-01 00:00:00
abstract::Preterm birth is an important cause of perinatal brain injury (PBI). Neurological injury in extremely preterm infants often begins in utero with chorioamnionitis (CHORIO) or inflammation/infection of the placenta and concomitant placental insufficiency. Studies in humans have shown dysregulated inflammatory signaling ...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000497273
更新日期:2019-03-28 00:00:00
abstract::A total of 216 male and female albino Wistar rats (Rattus norvegicus) between 10 and 60 days old from 42 litters were studied to determine mast cell characteristics within the thalamus. Whereas the mean numbers of mast cells within both the parenchyma and leptomeninges (combined) did not change appreciably after 15 da...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112759
更新日期:1981-01-01 00:00:00
abstract::Perinatal hypoxic ischemic (HI) brain injury is a leading cause of long-term neurological handicap in newborn babies. Recently, excessive activity of matrix metalloproteinases (MMPs), and in particular MMP-9, has been implicated in the aetiology of HI injuries to the immature brain. Our previous study suggested that M...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000343257
更新日期:2012-01-01 00:00:00
abstract::Cortex cerebri pieces from fetal donors were homologously and bilaterally grafted to the anterior eye chamber of adult albino rats. After intraocular maturation, presence of an external glial membrane, glial membranes surrounding blood vessels and cortical astrocytes was demonstrated using antisera raised against glia...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112703
更新日期:1982-01-01 00:00:00
abstract::The proximodistal axonal transport of choline acetyltransferase (ChAc) has been studied in the chick sciatic nerve in the absence of any experimental manipulation. The phenomenon utilized by us is a physiological transient fall in the activity of ChAc. This fall occurs in most areas of the chick central nervous system...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112484
更新日期:1979-01-01 00:00:00
abstract::This study investigated the time of expression of two endothelial cell adhesion molecules, ICAM-1/CD54 and PECAM-1/CD31 in the developing postpartum mouse blood-brain barrier (BBB). Immunoultrastructural studies demonstrated that both adhesion molecules are initially expressed primarily on the luminal endothelial cell...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000017352
更新日期:1998-01-01 00:00:00
abstract::In this study we have examined the susceptibility of the mitochondrial respiratory chain of astrocytes and astrocytes depleted of glutathione to peroxynitrite exposure. Astrocytes, as reported previously by us, appeared resistant to the actions of peroxynitrite. In contrast, depletion (-94%) of astrocytic glutathione ...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000111432
更新日期:1996-01-01 00:00:00
abstract::The cerebromicrovasculature of immature mice ranging in age from 1-24 days after birth was studied by electron microscopy. A micromanipulator apparatus enabled us to facilitate direction of a needle cannula and injection into leg veins or hearts with horseradish peroxidase (HRP) or ferritin (anionic and cationic) trac...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112242
更新日期:1986-01-01 00:00:00
abstract::In order to investigate the possible influence of norepinephrine (NE) on neuronal development, the effects of neonatal NE depletion on postnatally developing cell populations were characterized in (1) the normal mouse cerebellum, and (2) a cerebellar model system with delayed germinal cell development produced by neon...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112695
更新日期:1982-01-01 00:00:00
abstract::Insulin binding was measured on crude mitochondrial or plasma membranes prepared from different rat brain regions during postnatal development. In the cerebral cortex and brainstem, insulin binding decreased 60-70% between birth and the adult period. In the cerebellum, insulin binding doubled in the first 10 postnatal...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000111863
更新日期:1990-01-01 00:00:00
abstract::NeuroD is a basic helix-loop-helix (bHLH) transcription factor cloned from a two hybrid screen designed to search for new bHLH proteins. In our previous studies, we showed that NeuroD could convert Xenopus ectoderm into fully differentiated neurons and that it could prematurely differentiate neural precursor cells in ...
journal_title:Developmental neuroscience
pub_type: 杂志文章,评审
doi:10.1159/000111182
更新日期:1997-01-01 00:00:00
abstract::Neurite outgrowth is dependent on the presence in neurites of assembled microtubules which consist of polymerized tubulin to which microtubule-associated proteins (MAPs) are bound. This study adds to previous evidence of the similarity between the high molecular weight MAPs, MAP1X, MAP1B and MAP5, and explains the dif...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000111318
更新日期:1993-01-01 00:00:00
abstract::The kinetics and sodium-dependence of gamma-aminobutyric acid (GABA), hypotaurine and taurine uptake were analyzed in cerebral slices prepared from mice of different postnatal ages. The maximal velocities of transport (V) and the Km constants for the high-affinity uptake all decreased with age, whereas the development...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112354
更新日期:1983-01-01 00:00:00
abstract::There are distinct symptoms for attention deficit hyperactivity disorder (ADHD) at different ages. To explore the developmental mechanism of ADHD from childhood to adolescence, patients from different age groups with ADHD drawn from a large dataset should be investigated. In this study, we hypothesized that there are ...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000490289
更新日期:2018-01-01 00:00:00
abstract::Maternal infection during pregnancy increases the risk of neurobehavioral problems in offspring. Evidence from rodent models indicates that the maternal immune response to infection can alter fetal brain development, particularly in the hippocampus. However, information on the effects of maternal viral infection on fe...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000486850
更新日期:2018-01-01 00:00:00
abstract::Transient early cerebral laminar organization resulting from normal developmental events has been revealed in human beings through histology and imaging studies. DTI studies have postulated that the fractional anisotropy (FA)-based differentiation of different laminar structures reflects both differing cellular densit...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000229500
更新日期:2009-01-01 00:00:00
abstract::A leading hypothesis of the cause of neuronal death in Huntington's disease (HD) is excitotoxicity, in which subpopulations of striatal neurons are hypersensitive to glutamate release due to changes in postsynaptic N-methyl-D-aspartate receptors (NMDARs). In the present study we used RT-PCR methods on single cells and...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000091921
更新日期:2006-01-01 00:00:00
abstract::In some cells, the development of voltage-gated channels requires synaptic input, while in others it does not. Here we investigate whether the sodium and potassium currents in the skeletal muscle of zebrafish sofa potato (sop(-/-)) mutants develop normally. Zebrafish sop(-/-) mutants do not express nicotinic acetylcho...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000207794
更新日期:2009-01-01 00:00:00