Abstract:
:Maple Syrup Urine Disease (MSUD) is biochemically characterized by elevated levels of leucine, isoleucine and valine, as well as their corresponding transaminated branched-chain α-keto acids in tissue and biological fluids. Neurological symptoms and cerebral abnormalities, whose mechanisms are still unknown, are typical of this metabolic disorder. In the present study, we evaluated the early effects (1 h after injection) and long-term effects (15 days after injection) of a single intracerebroventricular administration of α-ketoisocaproic acid (KIC) on oxidative stress parameters and cognitive and noncognitive behaviors. Our results showed that KIC induced early and long-term effects; we found an increase in TBARS levels, protein carbonyl content and DNA damage in the hippocampus, striatum and cerebral cortex both one hour and 15 days after KIC administration. Moreover, SOD activity increased in the hippocampus and striatum one hour after injection, whereas after 15 days, SOD activity decreased only in the striatum. On the other hand, KIC significantly decreased CAT activity in the striatum one hour after injection, but 15 days after KIC administration, we found a decrease in CAT activity in the hippocampus and striatum. Finally, we showed that long-term cognitive deficits follow the oxidative damage; KIC induced impaired habituation memory and long-term memory impairment. From the biochemical and behavioral findings, it we presume that KIC provokes oxidative damage, and the persistence of brain oxidative stress is associated with long-term memory impairment and prepulse inhibition.
journal_name
Metab Brain Disjournal_title
Metabolic brain diseaseauthors
Taschetto L,Scaini G,Zapelini HG,Ramos ÂC,Strapazzon G,Andrade VM,Réus GZ,Michels M,Dal-Pizzol F,Quevedo J,Schuck PF,Ferreira GC,Streck ELdoi
10.1007/s11011-017-0035-zsubject
Has Abstractpub_date
2017-10-01 00:00:00pages
1507-1518issue
5eissn
0885-7490issn
1573-7365pii
10.1007/s11011-017-0035-zjournal_volume
32pub_type
杂志文章abstract::The effect of factors released from N2A neuroblastoma cells on the expression of myelin protein genes in glioma C6 cells, i.e., proteolipid protein (PLP) and myelin-associated glycoprotein (MAG), was studied. Both cells lines were propagated in serum-free DMEM-F10 (1:1) medium. The addition of 50% N2A conditioned medi...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF01000160
更新日期:1992-09-01 00:00:00
abstract::Levels of high mobility group box 1 (HMGB1), an important inflammatory mediator, are high in the serum of febrile seizure (FS) patients. However, its roles in FS and secondary epilepsy after prolonged FS are poorly understood. We demonstrate HMGB1's role in the pathogenesis of hyperthermia-induced seizures (HS) and se...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0103-4
更新日期:2017-12-01 00:00:00
abstract::Hepatic encephalopathy (HE) is a major complication of liver failure/disease which frequently develops during the progression of end-stage liver disease. This metabolic neuropsychiatric syndrome involves a spectrum of symptoms, including cognition impairment, attention deficits and motor dysfunction which eventually c...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-016-9843-9
更新日期:2016-12-01 00:00:00
abstract::Multiple sclerosis (MS) is a demyelinating and degenerative disease of the central nervous system (CNS) with a strong inflammatory component that affects more than 2 million people worldwide (and at least 400,000 in the United States). In MS, macrophage migration inhibitory factor (MIF) and D-dopachrome tautomerase (D...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-018-0331-2
更新日期:2019-02-01 00:00:00
abstract::There is evidence to suggest that, in acute liver failure (ALF), brain ammonia and proinflammatory cytokines may act synergistically to cause brain edema and its complications (intracranial hypertension, brain herniation). However, the molecular mechanisms involved remain to be established. In order to address this is...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-010-9185-y
更新日期:2010-03-01 00:00:00
abstract::Following axonal interruption, structural, metabolic and physiological parameters change in motorneurons. Also, glial cells are involved in this process. Microglia proliferate and express new proteins such as vimentin or MHC antigens. Astrocytes show hypertrophy, increased GFAP synthesis, and formation of lamellae. Bo...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/BF00999498
更新日期:1989-03-01 00:00:00
abstract::Stroke is devastating and a leading cause of morbidity and mortality worldwide. Cerebral ischemia-reperfusion and its subsequent reactive hyperemia lead to neuronal damage in the hippocampus and cognitive decline. Chrysin (5, 7-dihydroxyflavone) is a well-known member of the flavonoid family with antioxidant and neuro...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-019-00527-9
更新日期:2020-02-01 00:00:00
abstract::Schizophrenia (SCZ) and bipolar disorder (BD) are severe psychiatric disorders that share many genetic risk factors. This study aimed to investigate the association of phosphoinositide-3-kinase regulatory subunit1 (PIK3R1) gene rs3756668 and rs3730089 polymorphisms with SCZ and BD risks and determine the expression le...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-020-00552-z
更新日期:2020-06-01 00:00:00
abstract::Hyperphenylalaninemia (HPA) leads to increased oxidative stress in patients with phenylketonuria (PKU) and in animal models of PKU. Early diagnosis and immediate adherence to a phenylalanine-restricted diet prevents HPA and, consequently, severe brain damage. However, treated adolescent and adult PKU patients have dif...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-013-9414-2
更新日期:2013-12-01 00:00:00
abstract::Over the past 35 years, the outlook for a patient presenting with acute liver failure (ALF) has changed beyond all recognition. A patient presenting in 1984 had an 80 % likelihood of succumbing to intracranial hypertension. Today due to dramatic improvements in intensive care in dedicated liver transplant units, this ...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-012-9363-1
更新日期:2013-03-01 00:00:00
abstract::Patients with chronic liver disease manifest a high incidence (> 75%) of pallidal signal hyperintensity on T1-weighted Magnetic Resonance Imaging (MRI), the intensity of which correlates with blood manganese levels and the presence of extrapyramidal symptoms. A major cause of pallidal hyperintensity on T1-weighted MRI...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/BF02109357
更新日期:1995-12-01 00:00:00
abstract::To characterize an Egyptian patient with glutaric acidemia type I (GA I) and to identify the causative mutation(s) that may be responsible for the disease phenotype. MRI was performed on the patient using the 1.5 T magnet, biochemical analysis was carried out using gas chromatography/mass spectrometry on the patient's...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9879-x
更新日期:2017-02-01 00:00:00
abstract::Menkes disease is a very rare X-linked copper metabolism disorder that results from an ATP7A gene mutation. With the advent of subcutaneous copper-histidine therapy, the early diagnosis of Menkes disease becomes of utmost importance for patients' prognosis. In the present study, the clinical characteristics of 12 Kore...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9569-5
更新日期:2015-02-01 00:00:00
abstract::The present experiments were conducted to investigate the direct effects of ethanol on the energy metabolism of astrocytes and C6-glioma cells. Primary astrocytes were prepared from cerebral cortices of neonatal rats, and C6-glioma cells were purchased from American Type Culture Collection (ATCC). These cells were exp...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1023/a:1020606607729
更新日期:1999-09-01 00:00:00
abstract::Hepatic encephalopathy (HE) represents a nervous system disorder caused due to liver dysfunction. HE is broadly classified as acute/overt and moderate-minimal HE. Since HE syndrome severely affects quality of life of the patients and it may be life threatening, it is important to develop effective therapeutic strategy...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9556-x
更新日期:2014-12-01 00:00:00
abstract::Today, due to technology development and aversive events of daily life, Human exposure to both radiofrequency and stress is unavoidable. This study investigated the co-exposure to repeated restraint stress and WiFi signal on cognitive function and oxidative stress in brain of male rats. Animals were divided into four ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0016-2
更新日期:2017-10-01 00:00:00
abstract::The effects of chronic and repeated radiofrequency electromagnetic radiation (RFEMR) exposure on spatial cognition and hippocampal architecture were investigated in prepubescent rats. Four weeks old male Wistar rats were exposed to RF-EMR (900 MHz; SAR-1.15 W/kg with peak power density of 146.60 μW/cm(2)) for 1 h/day,...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-015-9689-6
更新日期:2015-10-01 00:00:00
abstract::Hereditary tyrosinemia type I (HT1) is caused by mutations in the fumarylacetoacetate hydrolase (FAH) gene, the template for the final enzyme in the tyrosine catabolism pathway. If left untreated this deficiency of functional FAH leads to a buildup of toxic metabolites that can cause liver disease, kidney dysfunction ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0071-8
更新日期:2017-12-01 00:00:00
abstract::Undernourished suckling rats were administered, by gastric intubation, either soy oil (which is rich in both linoleic and linolenic acids) or safflower oil (which is rich in linoleic acid but deficient in linolenic acid) to determine (1) if dietary supplementation would offset the hypomyelination characteristic of the...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF00999385
更新日期:1986-06-01 00:00:00
abstract::The aim of the present work was to investigate the role of ondansetron on the high fat diet (HFD) induced obese mice for behavioral and biochemical alterations using chronic unpredictable mild stress (CUMS) model of depression. Animals were fed with high fat diet for 14 weeks and subjected to different stress procedur...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9574-8
更新日期:2014-09-01 00:00:00
abstract::Tyrosinemia type II is an inborn error of metabolism caused by a deficiency in hepatic cytosolic aminotransferase. Affected patients usually present a variable degree of mental retardation, which may be related to the level of plasma tyrosine. In the present study we evaluated effect of chronic administration of L-tyr...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9615-3
更新日期:2015-02-01 00:00:00
abstract::It has been suggested that alterations of GABAergic neurotransmission are implicated in the pathophysiology of hepatic encephalopathy (HE). Increased concentrations of endogenous benzodiazepines with positive allosteric modulatory properties at the GABA-A receptor complex were proposed as a pathophysiological mechanis...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1023/b:mebr.0000043974.89820.22
更新日期:2004-12-01 00:00:00
abstract::Increased benzodiazepine (BZ) receptor density has been reported in brains of rabbits with hepatic encephalopathy (HE) due to galactosamine (GalN)-induced fulminant hepatic failure (FHF). These data were generated using detergent-Triton X-100-treated neural membranes. While performing further studies it was noted that...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF01000296
更新日期:1989-09-01 00:00:00
abstract::Mobile phone providers use electromagnetic radiation (EMR) with frequencies ranging from 900 to 1800 MHz. The increasing use of mobile phones has been accompanied by several potentially pathological consequences, such as neurological diseases related to hippocampal (HIPPON) and dorsal root ganglion neuron (DRGN). The ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0180-4
更新日期:2018-06-01 00:00:00
abstract::Maple syrup urine disease (MSUD) or branched-chain alpha-keto aciduria (BCKA) is an inherited disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKAD) activity. The blockage of this pathway leads to tissue accumulation of the branched-chain amino acids (BCAA) leucine, isoleuc...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-006-9030-5
更新日期:2006-12-01 00:00:00
abstract::Stroke is an increasingly prevalent clinical condition and second leading cause of death globally. The present study evaluated the therapeutic potential of Indian Ginseng, also known as Withania somnifera (WS), supplementation on middle cerebral artery occlusion (MCAO) induced mitochondrial dysfunctions in experimenta...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-018-0234-2
更新日期:2018-08-01 00:00:00
abstract::Effects of sertraline, haloperidol or olanzapine administration on ketamine-induced behaviours in mice were examined. The aim was to ascertain the degree of reversal of such behaviours by sertraline, and compare its effectiveness to haloperidol and olanzapine. Ten-week old mice (N = 120) were equally divided into main...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0031-3
更新日期:2017-10-01 00:00:00
abstract::The free radical scavenging abilities of the structurally related steroids beta-sitosterol, beta-sitosterol glucoside (plant sterols and sterolins), cholesterol, and dehydroepiandrosterone sulphate (DHEAS) were compared with melatonin (an efficient free radical scavenger) in an in vitro system which measures lipid per...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1023/a:1011167023695
更新日期:2000-12-01 00:00:00
abstract::The aim of this study was to explore the neuroprotective effects of active compounds from Schisandra chinensis (Trucz.) Baill. (Magnoliaceae) against the D-galactose (D-gal)-induced neurotoxicity in rat. The Wistar rats were subcutaneously injected with D-gal (150 mg/(kg day)) for six weeks and orally administered wit...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9804-3
更新日期:2016-06-01 00:00:00
abstract::Chronic neuroinflammation is strongly associated with AD and altered peripheral and central levels of chemokines and cytokines have been frequently described in those with AD. Given the increasing evidence of ethnicity-related differences in AD, it was of interest to determine if those altered chemokine and cytokine l...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-019-00512-2
更新日期:2020-01-01 00:00:00