Abstract:
:Tyrosinemia type II is a genetic disorder characterized by elevated blood levels of the amino acid tyrosine caused by the deficiency of tyrosine aminotransferase enzyme, resulting in neurologic and developmental difficulties in the patients. Although neurological sequelae are common in Tyrosinemia type II patients, the mechanisms involved are still poorly understood. The oxidative stress appears to be, at least in part, responsible for neurological complication in this inborn error metabolism. We observed that an acute injection of tyrosine in rats caused a massive oxidative stress in different brain structures. The glutathione system and superoxide dismutase enzyme are relevant antioxidant strategies of the cells and tissues, including in the brain. Other important point is the strong relation between oxidative damage and inflammatory events. Herein, we investigated the effects of chronic administration of tyrosine in the hippocampus of young rats, with emphasis in the activity of GSH related enzymes and superoxide dismutase enzyme, and the astrocytosis. We observed that rats exposed to high levels of tyrosine presented an increased content of tyrosine, which was associated with an increment in the activity of glutathione peroxidase and glutathione reductase as well as with a diminished activity of superoxide dismutase. This antioxidant imbalance was accompanied by enhanced glial fibrillary acidic protein immunoreactivity, a marker of astrocytes, in the brain area studied. In conclusion, hippocampus astrogliosis is also a characteristic of brain alteration in Tyrosinemia. In addition, the chronic exposition to high levels of tyrosine is associated with an alteration in the activity of fundamental antioxidant enzymes.
journal_name
Metab Brain Disjournal_title
Metabolic brain diseaseauthors
Carvalho-Silva M,Gomes LM,de Prá SD,Wessler LB,Schuck PF,Scaini G,de Bem AF,Blum-Silva CH,Reginatto FH,de Oliveira J,Streck ELdoi
10.1007/s11011-019-00511-3subject
Has Abstractpub_date
2020-01-01 00:00:00pages
193-200issue
1eissn
0885-7490issn
1573-7365pii
10.1007/s11011-019-00511-3journal_volume
35pub_type
杂志文章abstract::This study aimed to examine the neuroprotective effects of Nigella sativa (N. sativa) in the hippocampus of propylthiouracil (PTU)-induced hypothyroid rats during neonatal and juvenile growth. Twenty- five pregnant rats from early gestation (GD 0) were divided into five groups: (1) control (received drinking water), (...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0025-1
更新日期:2017-10-01 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::This study was performed to analyze the mutational spectrum of the phenylalanine hydroxylase (PAH) gene in phenylketonuria (PKU) patients in Northwest China, to identify mutational hot spots, and to determine the correlation between variants and clinical phenotypes of PKU. A large cohort of 475 PKU families in Northwe...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-019-0387-7
更新日期:2019-06-01 00:00:00
abstract::Neuroinflammation has been recognized as a major contributor to brain injury caused by intracerebral hemorrhage (ICH). Nucleotide-binding oligomerization domain-like receptor family pyrin domain-containing 3 (NLRP3) inflammasome acts as an important mediator of inflammatory response in various inflammation-related dis...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0003-7
更新日期:2017-08-01 00:00:00
abstract::In the normal adult brain, glucose provides 90% of the energy requirements as well as substrate for nucleic acid and lipid synthesis. In the present study, effects of ethanol on glucose transporters (GLUT) and glucose utilization were examined in rat brain. Male Sprague-Dawley rats weighing 250-300 gms were given eith...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF02674530
更新日期:2000-09-01 00:00:00
abstract::The enzyme nitric oxide synthase has been associated with suicide behavior. NOS1, NOS2 and NOS3 genes are implicated in the production of nitric oxide. However, the association between NOS genes and suicide behavior has not yet been established. To assess the association of Nitric Oxide Synthase (NOS) genes and suicid...
journal_title:Metabolic brain disease
pub_type: 杂志文章,meta分析
doi:10.1007/s11011-019-00406-3
更新日期:2019-08-01 00:00:00
abstract::Intracerebroventricular (ICV) injection of streptozotocin (STZ) is a well established procedure to induce neuroinflammation leading to dementia in experimental animals. However, the optimal dose of STZ has not been determined. In the present study, rats were ICV injected with 1.5, 3 and 6 mg of STZ per kg of body weig...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-020-00588-1
更新日期:2020-12-01 00:00:00
abstract::Rare metabolic diseases may sometimes arise acutely and endanger human life if not immediately recognized and treated. Marchiafava Bignami disease is an uncommon neurologic disorder described in alcohol abusers and characterized by an acute severe damage of brain white matter. Even more rarely, it has been reported in...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9893-z
更新日期:2017-02-01 00:00:00
abstract::As feared and deadly human diseases globally, Rabies virus contrived mechanisms to escape early immune recognition via suppression of the interferon response. This study, preliminarily investigated whether Rabies virus employs epigenetic mechanism for the suppression of the interferon using the Challenge virus standar...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-020-00553-y
更新日期:2020-06-01 00:00:00
abstract:PURPOSE:To analyze brain metabolite changes in HIV-1-seropositive subjects in order to define whether the neuronal impairment is a localized or more diffuse process. MATERIALS AND METHODS:15 patients and 18 volunteers underwent multivoxel proton magnetic resonance (MR) spectroscopy at 1.5T. Nine patients were classifi...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1023/a:1020609213664
更新日期:1998-06-01 00:00:00
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::We report treatment outcome of eleven patients with pyridoxine-dependent epilepsy caused by pathogenic variants in ALDH7A1 (PDE-ALDH7A1). We developed a clinical severity score to compare phenotype with biochemical features, genotype and delays in the initiation of pyridoxine. Clinical severity score included 1) globa...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9933-8
更新日期:2017-04-01 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::The commonly used volatile anesthetic sevoflurane has been shown to induce widespread apoptosis in the developing brain, yet the underlying molecular mechanisms are not fully understood. Accumulating research has demonstrated that long noncoding RNAs (lncRNAs) regulate multiple biological processes, including neural d...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9838-6
更新日期:2016-10-01 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::Lead exposure has negative effects on developing nervous system and induces apoptosis in newly generated neurons. Natural antioxidants (i.e. Ascorbic acid and Garlic) might protect against lead-induced neuronal cell damage. The aim of the present study was to investigate the protective effects of Ascorbic acid and Gar...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9837-7
更新日期:2016-10-01 00:00:00
abstract::Phenylketonuria (PKU) is the most common inborn error of amino acid metabolism. Usually diagnosed within the first month of birth, it is essential that the patient strictly follow the dietary restriction of natural protein intake. Otherwise, PKU impacts the development of the brain severely and may result in microceph...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-019-00472-7
更新日期:2019-12-01 00:00:00
abstract::Acute and chronic administration of Al-gluconate (12.7% Al) at the concentration of 1 mg/kg produces edema in the rat brain, as reflected by the increase in water and Na+ content. The permeability for Evans blue is also increased, which indicates the opening of the blood-brain barrier. Higher concentrations of the Al-...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF00996974
更新日期:1990-03-01 00:00:00
abstract::Alzheimer's Disease (AD) is a complex neurodegenerative disorder often associated with aging and characterized by several critical molecular changes that take place in the brain. Among the molecular hallmarks of AD, increased levels of amyloid β-peptide (Aβ) and the subsequent Aβ-derived damage are the most well-studi...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-014-9527-2
更新日期:2014-09-01 00:00:00
abstract::Autism (MIM 209850) is a heterogeneous neurodevelopmental disease that manifests within the first 3 years of life. Numerous articles reported that dysfunctional folate-methionine pathway enzymes may play an important role in the pathophysiology of autism. Methylenetetrahydrofolate reductase (MTHFR) is a critical enzym...
journal_title:Metabolic brain disease
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s11011-016-9815-0
更新日期:2016-08-01 00:00:00
abstract::The lifetime prevalence rate for major depressive disorder (MDD) is approximately 17 % for most developed countries around the world. Dietary polyphenols are currently used as an adjuvant therapy to accelerate the therapeutic efficacy on depression. Ferulic acid (FA) or 4-hydroxy-3-methoxy-cinnamic acid (Fig. 1a) is a...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-015-9704-y
更新日期:2015-12-01 00:00:00
abstract::Traumatic brain injury (TBI) is accompanied with enhanced matrix metalloproteinase-9 (MMP-9) activity and elevated levels of plasma fibrinogen (Fg), which is a known inflammatory agent. Activation of MMP-9 and increase in blood content of Fg (i.e. hyperfibrinogenemia, HFg) both contribute to cerebrovascular disorders ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9550-3
更新日期:2015-04-01 00:00:00
abstract::Current data on the neurotrophic effects of steroid hormones suggest that, in brain and spinal cord regions containing receptor systems, steroids act at the level of RNA and protein synthesis to effect metabolic changes associated with nerve-cell survival, elaboration/maintenance of dendritic and axonal processes, syn...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/BF01001350
更新日期:1988-03-01 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::Although the key contributors of altering neurological function in hepatic encephalopathy are relatively well known, the electrophysiological mechanism of CA1 damage, a key vulnerable area during hyperammonemia, have not yet been defined. Therefore, here we focus on the electrophysiological mechanisms of cognitive imp...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-9966-7
更新日期:2017-06-01 00:00:00
abstract::Chronic exposure to stress amplifies locomotor deficits and exacerbates dopamine neuron loss in an animal model for Parkinson's disease. The release of neurotrophic factors such as glial cell-line derived neurotrophic factor (GDNF) and neurotrophin-3 (NT-3) following neuronal injury attenuates exacerbated degeneration...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-9988-1
更新日期:2017-08-01 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::Homocystinuria is a metabolic disorder caused by deficiency of cystathione beta-synthase activity leading to tissue accumulation of homocysteine (Hcy); affected patients present neurological dysfunction. Considering that Hcy induces free radical formation and that memory is impaired by oxidative stress, in the present...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1023/a:1019982223034
更新日期:2002-09-01 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::Statins or HMG-CoA reductase inhibitors have been shown to be effective at lowering cholesterol levels, and the application of these molecules has gradually emerged as an attractive therapeutic strategy for neurodegenerative diseases. Epidemiological studies suggest that statin use is associated with a decreased incid...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0091-4
更新日期:2017-12-01 00:00:00