Abstract:
:Cystinosis is a systemic genetic disease caused by a lysosomal transport deficiency accumulating cystine in most tissues. Tissue damage depends on cystine accumulation, but the mechanisms of this damage are still obscure. Cysteamine administration depletes cystine accumulated, increasing survive of affected patients. Studies performed in fibroblasts of cystinotic patients suggest that apoptosis is enhanced in this disease. Considering that oxidative stress is a known apoptosis inducer, our main objective was to investigate a possible antioxidant effect of cysteamine on several parameters of oxidative stress in the brain of young rats. Animals received three subcutaneous injections at 3-h intervals of a buffered solution (pH 7.4) of 10 mg/kg body weight cysteamine and were sacrificed 1 h after the last injection. Cysteamine decreased lipoperoxidation and glutathione peroxidase activity, and increased the carbonyl content of proteins and catalase activity. In vitro studies showed that cysteamine reduced lipoperoxidation, 2',7'-dihydrodichlorofluorescein oxidation, carbonyl content of proteins and catalase activity, and increased glutathione peroxidase activity. These results suggest that cysteamine may act as a scavenger of superoxide free radicals and hydrogen peroxide. Therefore, it is possible that cysteamine may extend life of cystinotic patients acting not only as a cystine depleting drug, but also as a free radical scavenger, reducing cell damage by apoptosis.
journal_name
Metab Brain Disjournal_title
Metabolic brain diseaseauthors
Kessler A,Biasibetti M,Feksa LR,Rech VC,Melo DA,Wajner M,Dutra-Filho CS,Wyse AT,Wannmacher CMdoi
10.1007/s11011-007-9078-xsubject
Has Abstractpub_date
2008-03-01 00:00:00pages
81-93issue
1eissn
0885-7490issn
1573-7365journal_volume
23pub_type
杂志文章abstract::Mitochondrial glutathione (mGSH) is a critical factor in the cell defense against oxidative and nitrosative stress (ONS), and ONS is a key pathogenic event in hepatic encephalopathy (HE). Acute HE in the thioacetamide (TAA) model caused a 54 % decrease of mGSH content in the rat prefrontal cortex (pfc), but not in the...
journal_title:Metabolic brain disease
pub_type: 杂志文章
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abstract::The sprouting of new blood vessels from pre-existing vasculature (angiogenesis) is essential for tumour survival, influenced by tumour cell-endothelial cell interactions and is tightly regulated by biochemical cues including the kallikrein-kinin system (KKS). We examined the structural interaction between neuroblastom...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-006-9008-3
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journal_title:Metabolic brain disease
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abstract::Stroke is the leading cause of disability among adults as well as the 2nd leading cause of death globally. Ischemic stroke accounts for about 85% of strokes, and currently, tissue plasminogen activator (tPA), whose therapeutic window is limited to up to 4.5 h for the appropriate population, is the only FDA approved dr...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
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abstract::Microglia--the macrophage equivalent of the CNS--safeguards and supports neuronal functions. Threats to the CNS homeostasis can trigger a rapid transformation of these cells from a normally "resting" into alerted and "activated" states. Microglia primarily serves the tissue defence and protection when participating in...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
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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, th...
journal_title:Metabolic brain disease
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doi:10.1007/s11011-019-00511-3
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abstract::The synthesis, storage and release of acetylcholine (ACh) requires the expression of several specialized proteins, including choline acetyltransferase (ChAT) and the vesicular ACh transporter (VAChT). The VAChT gene is located within the first intron of the ChAT gene. This unique genomic organization permits coordinat...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
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journal_title:Metabolic brain disease
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doi:10.1007/s11011-013-9416-0
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journal_title:Metabolic brain disease
pub_type: 历史文章,杂志文章
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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
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abstract::In the brain hyperammonemia interferes with ion homeostasis, membrane potentials, neurotransmission, and neurotransmitter recycling and reduces metabolic rates for oxygen and glucose. Because, cerebral blood flow (CBF) is closely coupled to metabolism, CBF is most often reduced in diseases associated with hyperammonem...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
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更新日期:2002-12-01 00:00:00
abstract::The radiological spectrum of neuromyelitis optica has become broader since the detection of aquaporin4 antibodies. We report a case of neuromyelitis optica patient with pseudotumoral encephalic lesion. A 66 year-old woman presented with sudden left lateral homonymous hemianopsia. A brain MRI showed an isolated and ext...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0141-y
更新日期:2018-02-01 00:00:00
abstract::Maternal separation in non-human primates has been proposed as a model of early adversity. The symptoms of separation anxiety were studied in vervet monkeys, during the weaning period, when psychotropic medications were administered. The control group received a normal diet and treatment groups received citalopram, re...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-006-9011-8
更新日期:2006-09-01 00:00:00
abstract::Because of the importance of cholesterol metabolism in the physiopathogenesis of dementia, and knowing the function of ATP-binding cassette A1 transporter (ABCA1) as a cholesterol flow pump at the cellular and cerebral level, it has been noted that the ABCA1 gene may be a good candidate for disease study. In order to ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-020-00577-4
更新日期:2020-08-01 00:00:00
abstract::Methamphetamine (METH) is a highly addictive psycho-stimulant that induces behavioral changes due to high level of METH-induced dopamine in the brain. Nucleus accumbens (NAc) plays an important role in these changes, especially in drug addiction. However, little is known about the underlying molecular mechanisms of ME...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0061-x
更新日期:2017-12-01 00:00:00
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journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-018-0262-y
更新日期:2018-10-01 00:00:00
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journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-005-7210-3
更新日期:2005-09-01 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
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更新日期:2016-08-01 00:00:00
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journal_title:Metabolic brain disease
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更新日期:2018-10-01 00:00:00
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journal_title:Metabolic brain disease
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更新日期:2014-09-01 00:00:00
abstract::The blood-brain barrier and the intestinal barrier show signs of disruption in patients with idiopathic Parkinson's disease (PD) and animal models of nigrostriatal degeneration, and likewise in amyotrophic lateral sclerosis (ALS) models. A substantial body of evidence shows that defects in epithelial membrane barriers...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-018-0239-x
更新日期:2018-08-01 00:00:00
abstract::Wilson disease (WD) is an autosomal recessive inherited disorder of copper metabolism, resulting in pathological accumulation of copper in many organs and tissues. The hallmarks of the disease are the presence of liver disease, neurologic symptoms, and Kayser-Fleischer corneal rings. The leading neurologic symptoms in...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-005-7910-8
更新日期:2005-12-01 00:00:00
abstract::Glutamate neurotoxicity was studied in primary neuronal cultures prepared from rat cerebral cortex and hippocampal CA1 sector. Neurons were cultivated with 5% native horse serum and then exposed to 0.1 or 1.0 mM glutamate for 5 min. Subsequently, neurons were allowed to recover for 24 hours either in the presence or i...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF02098880
更新日期:1994-12-01 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::Homocystinuria is an inborn error of sulfur amino acid metabolism characterized predominantly by vascular and nervous system dysfunction. In this study we determined the in vitro effects of homocysteine and methionine, metabolites which accumulate in homocystinuria, on Na+, K+-ATPase, and Mg2+-ATPase activities in syn...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1023/a:1015594111778
更新日期:2002-06-01 00:00:00
abstract::We discuss the case of a rare and often unrecognized neurologic syndrome, called Acquired Hepatocerebral Degeneration (AHD), observed in patients with advanced liver disease and portosystemic shunts. The clinical manifestations can be very heterogeneous and in our case included a combination of cerebellar and extrapyr...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0107-0
更新日期:2018-02-01 00:00:00
abstract::Susceptibility to Wilson's disease (WD) and its clinical manifestations are thought to be affected by genetic factors, including polymorphisms. The role of brain-derived neurotrophic factor (BDNF) in the pathogenesis of neurodegenerative diseases is now widely discussed. The aim of the present study was to evaluate th...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-013-9399-x
更新日期:2013-09-01 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::The present study analyses the potential role of MTHFR gene polymorphism, folate supplementation and dietary pattern among the mothers of NTD neonates and controls in heterogeneous populations of North India, with the special focus on their ethnic labels. Results indicated significant increased risk for neural tube de...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-011-9256-8
更新日期:2011-09-01 00:00:00