Abstract：
:The succinic semialdehyde dehydrogenase (SSADH) null mouse (SSADH(-/-)) represents a viable animal model for human SSADH deficiency and is characterized by markedly elevated levels of both gamma-hydroxybutyric acid (GHB) and gamma-aminobutyric acid (GABA) in brain, blood, and urine. In physiological concentrations, GHB acts at the GHB receptor (GHBR), but in high concentrations such as those observed in the brains of children with SSADH deficiency, GHB is thought to be a direct agonist at the GABABR receptor (GABABR). We tested the hypothesis that both GHBR and GABABR-mediated function are perturbed in SSADH deficiency. Therefore, we examined the high affinity binding site for GHB as well as the expression and function of the GABABR in mutant mice made deficient in SSADH (SSADH(-/-)). There was a significant decrease in binding of the specific GABABR antagonist, [3H]CGP-54626A at postnatal day (PN)7 and PN14 in SSADH(-/-) when compared to wild type control animals (SSADH(+/+)), particularly in hippocampus. GABABR-mediated synaptic potentials were decreased in SSADH(-/-). Immunoblot analysis of GABABR1a, R1b, and R2 in SSADH(-/-) indicated a trend towards a region-specific and time-dependent decrease of GABABR subunit protein expression. There was no difference between SSADH(-/-) and wild type in binding of either [3H]GHB or a specific GHBR antagonist to the GHBR. These data suggest that the elevated levels of GABA and GHB that occur in SSADH(-/-) lead to a use-dependent decrease in GABABR-mediated function and raise the possibility that this GHB- and GABA-induced perturbation of GABABR could play a role in the pathogenesis of the seizures and mental retardation observed in SSADH deficiency.
journal_name
Brain Resjournal_title
Brain researchauthors
Buzzi A,Wu Y,Frantseva MV,Perez Velazquez JL,Cortez MA,Liu CC,Shen LQ,Gibson KM,Snead OC 3rddoi
10.1016/j.brainres.2006.02.131subject
Has Abstractpub_date
2006-05-23 00:00:00pages
15-22issue
1eissn
0006-8993issn
1872-6240pii
S0006-8993(06)00599-3journal_volume
1090pub_type
杂志文章相关文献
BRAIN RESEARCH文献大全abstract：:While it is generally assumed that cocaine self-administration (SA) is determined and maintained by the pharmacological actions of cocaine in the brain, it is also a drug-motivated and drug-reinforced goal-directed behavior, which is determined by concurrent learning and behavioral performance. To dissociate the contr...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2004.01.038
更新日期：2004-04-16 00:00:00
abstract：:Adipose-derived stem cells (ASCs) are readily accessible multipotent mesenchymal stem cells and are known to secrete multiple growth factors, and thereby to have cytoprotective effects in various injury models. In the present study, the authors investigated the neuroprotective effect of ASCs in an intracerebral hemorr...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2007.09.005
更新日期：2007-12-05 00:00:00
abstract：:The cockroach can reduce the amount of sensory activity received by its CNS from the cerci, paired wind-responsive appendages. This reduction is produced by medial displacement of the cerci, a movement the animal performs naturally during flying. We demonstrate here that this sensory reduction significantly reduces ac...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/0006-8993(88)91009-8
更新日期：1988-02-09 00:00:00
abstract：:Individual neurons were recorded extracellularly in the nucleus accumbens (NAcc) of rats during cocaine self-administration sessions. NAcc neurons exhibited a variety of phasic changes in firing rate within the few seconds before and/or after cocaine self-infusion. Analysis of the topographical distribution of the pha...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/s0006-8993(97)00752-x
更新日期：1997-09-05 00:00:00
abstract：:The aim of this study was to describe the normal distribution of the water content in several regions of the rat brain and furthermore to determine if global ischemia results in a variable increase in water content in these regions. Water content was determined in 40 regions; in 8 unaffected brains by gravimetry and i...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/s0006-8993(96)01165-1
更新日期：1997-02-21 00:00:00
abstract：:This manuscript describes the use of staining of Fos-related antigens (FRAs) as markers for changes in neuronal activity. The model system consisted of the tuberoinfundibular dopamine (TIDA) neurons located in the arcuate nucleus of the hypothalamus. Under normal conditions, these neurons are devoid of c-Fos staining ...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/0006-8993(94)90481-2
更新日期：1994-08-22 00:00:00
abstract：:Intracellular and extracellular recordings from CA3 hippocampal neurons in vitro were used to study the ability of several NMDA (N-methyl-D-aspartate) receptor antagonists to suppress epileptiform bursts induced by NMDA and convulsants not thought to act at NMDA receptors. The antagonists, APV (D-2-amino-5-phosphonova...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/0006-8993(88)90642-7
更新日期：1988-09-06 00:00:00
abstract：:In the adult brain, γ-amino butyric acid (GABA) is an inhibitory neurotransmitter, whereas it acts as an excitatory transmitter in the immature brain, and may be involved in morphogenesis. In the present study, we immunohistochemically examined the developmental changes in GABA signaling in the embryonic mouse cervica...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2012.10.003
更新日期：2012-11-27 00:00:00
abstract：:Source memory for the speaker's voice (male or female) was investigated when semantic knowledge (gender stereotypes) could and could not inform the episodic source judgment while event-related potentials (ERPs) were recorded. Source accuracy was greater and response times were faster when stereotypes could predict the...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2012.11.013
更新日期：2013-01-23 00:00:00
abstract：:The time course of autoradiographic binding of major second messengers in the rat brain was studied at 2, 7, and 24 h after withdrawal from butorphanol infusion. [3H]Forskolin and [3H]phorbol 12,13-dibutyrate (PDBu) were used to label adenylate cyclase and protein kinase C (PKC), respectively. Rats were rendered depen...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/s0006-8993(99)01127-0
更新日期：1999-03-13 00:00:00
abstract：:As in many humans suffering from lupus erythematosus, the development of systemic autoimmunity and inflammation in Fas-deficient MRL-lpr mice is accompanied by CNS dysfunction of unknown etiology. Experimental studies revealed infiltration of lymphoid cells into the choroid plexus, reduced neuronal complexity, retarde...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/s0006-8993(02)03980-x
更新日期：2003-02-28 00:00:00
abstract：:Retrograde transport of horseradish peroxidase (HRP) was used to identify supraependymal neurons projecting to the pituitary gland in the hamster. Supraependymal neurons overlying the median eminence were labeled by HRP injections into the neural and intermediate lobes of the pituitary gland; no neurons were labeled f...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/0006-8993(89)91502-3
更新日期：1989-01-30 00:00:00
abstract：:Treatment with thyrotropin-releasing hormone (TRH) significantly improved survival following induction of fatal systemic anaphylaxis in mice. The protective effect was mediated centrally since survival was increased by intracerebroventricular (i.c.v.) administration of TRH at doses which had no effect when given syste...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/0006-8993(84)91421-5
更新日期：1984-04-30 00:00:00
abstract：:Intracranial pressure (ICP) was monitored to evaluate the therapeutic effect of hyperbaric oxygen (HBO(2)) treatment following traumatic brain injury (TBI). This subject is controversial. The aim of our study was to determine whether HBO(2) treatment has a therapeutic effect on ICP dynamics and survival following seve...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2005.02.049
更新日期：2005-06-21 00:00:00
abstract：:Fluoxetine is widely used for the treatment of depression. We examined the action of fluoxetine on cloned Kv4.3 stably expressed in CHO cells using the whole-cell patch-clamp technique. Fluoxetine did not significantly produce a reduction in the peak amplitude of Kv4.3, but increased the rate of current inactivation i...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2013.01.028
更新日期：2013-03-15 00:00:00
abstract：:Transgenic mice expressing human APPswe and PS1-A264E mutations mimic certain neuropathological features of Alzheimer's disease (AD). These mice have elevated levels of the highly fibrillogenic amyloid beta1-42 peptide (Abeta42) and develop amyloid plaques around the age of 9 months. Our aim was to find whether these ...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/s0006-8993(02)02617-3
更新日期：2002-07-12 00:00:00
abstract：:Interpretation of the cellular and molecular pathogenic basis of post-minimal traumatic brain injury is a significant clinical and scientific problem, especially due to the high prevalence of motor vehicle--and other accidents. Pathogenetic brain mechanisms following traumatic impact are usually investigated by using ...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2006.10.032
更新日期：2007-01-26 00:00:00
abstract：:Diabetic retinopathy is the leading cause of acquired blindness in working-age individuals. Recent work has revealed that neurodegeneration occurs earlier than vascular insult and that distal optic nerve damage precedes retinal degeneration and vascular insult. Since we have shown that optic nerve degeneration is redu...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2018.07.018
更新日期：2018-12-01 00:00:00
abstract：:To determine if aging in rats is associated with insensitivity of cerebral tissue to thyroid hormones (TH), the expression of a TH responsive protein or (THRP) in cerebral tissue was studied in male Fischer rats at 4, 12 and 24 months of age during euthyroid, hypothyroid and hyperthyroid states. The basal levels of TH...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/s0006-8993(98)00144-9
更新日期：1998-05-18 00:00:00
abstract：:The suprachiasmatic nucleus (SCN) of the anterior hypothalamus is implicated in the temporal organization of circadian rhythms in a variety of physiological, endocrine and behavioral processes. There is a great deal of evidence indicating that aging is characterized by a progressive deterioration of circadian timekeep...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/0006-8993(94)90689-0
更新日期：1994-07-18 00:00:00
abstract：:Previous studies have indicated that central sensitization is a state of increased excitability of nociceptive neurons in the spinal dorsal horn following peripheral tissue injury and/or inflammation and astrocytes play an important role in the central sensitization. The current study investigated the role of amino ac...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2012.02.018
更新日期：2012-04-17 00:00:00
abstract：:In rats with unilateral 6-hydroxydopamine lesions of the substantia nigra, a specific D1 dopamine receptor agonist, SKF 38393A, at a dose that does not itself produce turning, significantly increased the contralateral rotation observed following a low dose of the specific D2 agonist LY 171555. Doses of SKF 38393A or t...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/0006-8993(86)91178-9
更新日期：1986-10-08 00:00:00
abstract：:The neurobiology of social bonding, despite its relevance to human mental health, has been studied primarily in rodents. In this study we used position emission tomography (PET), registered with structural magnetic resonance imaging (MRI) to investigate central glucose uptake in 17 males of a monogamous primate specie...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2007.09.087
更新日期：2007-12-12 00:00:00
abstract：:The resurgent Na(+) current (I(NaR)) is a component of neuronal voltage-dependent Na(+) currents that is activated by repolarization and is believed to result from an atypical path of Na(+)-channel recovery from inactivation. So far, I(NaR) has only been identified in a small number of central neuronal populations in ...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2007.05.065
更新日期：2007-08-13 00:00:00
abstract：:Neurotensin-producing perikarya and fibers have been identified in the ventral tegmental area of the rat, and recent microinjection studies indicate that neurotensin may function in the ventral tegmental area to regulate body temperature. In this study, the hypothermic response produced by intraventral tegmental injec...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/0006-8993(85)90031-9
更新日期：1985-02-11 00:00:00
abstract：:Physical activity has been related to antioxidant adaptations, which is associated with health benefits, including those to the nervous system. Additionally, available data suggest exercise and a caloric restriction regimen may reduce both the incidence and severity of neurological disorders. Therefore, our aim was to...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2011.08.003
更新日期：2011-11-03 00:00:00
abstract：:Many lines of evidence support the importance of the nucleus accumbens (NAC) for ethanol-reinforced behavior. The nature of the neuronal activity that occurs in this region during ethanol self-administration is not known. We recorded from ensembles of single-units primarily located within the shell of the NAC during o...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/s0006-8993(98)01245-1
更新日期：1999-01-30 00:00:00
abstract：:Horseradish peroxidase was injected intracellularly into motoneurons responding to cutaneous stimulation of the central foot pad of the hind limb in cats. Three motoneurons were selected for detailed analysis: two excited by foot pad stimulation, and one postsynaptically inhibited by such stimulation. The overall leng...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/0006-8993(82)90669-2
更新日期：1982-12-16 00:00:00
abstract：:Studies evaluating the role of steroid hormones in sexual differentiation of the zebra finch song system have produced complicated and at times paradoxical results, and indicate that additional factors may be critical. Therefore, in a previous study we initiated a screen for differential gene expression in the telence...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2006.08.002
更新日期：2006-12-18 00:00:00
abstract：:Physical exercise can induce brain plasticity and reduce the cognitive decline observed in type 1 diabetes mellitus (T1DM). We investigated the effects of physical exercise to prevent or reverse spatial memory deficits produced by diabetes and some biochemical and immunohistochemical changes in hippocampal astrocytes ...
journal_title：Brain research
pub_type： 杂志文章
doi：10.1016/j.brainres.2016.10.024
更新日期：2017-01-15 00:00:00
