Abstract:
:Cerebral inflammation plays a role in diseases such as multiple sclerosis (MS), Alzheimer's disease (AD), and depression. Iron is involved in infection and inflammation through free radical production. Theoretically transferrin should prohibit iron from participating in oxidative reactions, but transferrin has also been found to promote free radical damage. We reported previously that isolation of transferrin from plasma by ion exchange column chromatography produced a broad pink protein band that subsequently separated on a gel filtration column into three proteins containing many metals. In this study some properties of the three proteins were studied in 20 volunteers. Protein 3 (identified as transferrin by nephelometry) contained the most iron while Protein 1 (called "toxiferrin") contained significantly less iron (p < 0.00001). Plasma from volunteers obtained under conditions of infection/inflammation with fever (n = 5) had a significantly increased toxiferrin to transferrin ratio compared to healthy volunteers (n = 15; p < 0.001). In vitro, Protein 2 and transferrin inhibited lipid peroxidation, while toxiferrin (possibly a protease degradation product of transferrin), enhanced lipid peroxidation. Also, toxiferrin (1 mg/mL) caused a significant increase in viability of monocytes as measured by the 3-(4,5-dimethyl-thiazol-2-yl) 2,5-diphenyl tetrazolium bromide (MTT) reduction test, as well as the morphological transformation of monocytes to macrophages.
journal_name
Metab Brain Disjournal_title
Metabolic brain diseaseauthors
van Rensburg SJ,van Zyl J,Hon D,Daniels W,Hendricks J,Potocnik F,Erasmus Rdoi
10.1023/b:mebr.0000027421.33085.8bsubject
Has Abstractpub_date
2004-06-01 00:00:00pages
97-112issue
1-2eissn
0885-7490issn
1573-7365journal_volume
19pub_type
杂志文章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::We investigated the biochemical alterations of the striatum of mice subjected to seven experimental schedules with 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine hydrochloride (MPTP) treatment. The mice were treated intraperitoneally (i.p.) with MPTP (20 mg/kg in saline) four times a day at 2-hr intervals showed severe ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-010-9195-9
更新日期:2010-06-01 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::In patients with hepatic encephalopathy (HE) the blood concentration of ammonia is usually highly elevated. Ammonia readily enters brain cells from the blood, and toxic effects of ammonia on brain metabolism and neurotransmission are believed to play a key role in the pathogenesis of HE. It has, however, been a matter...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-008-9126-1
更新日期:2009-03-01 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
abstract::Spontaneous intracerebral hemorrhage (ICH) represents a partially-understood cerebrovascular disease of high incidence, morbidity and mortality. We, herein, report the findings of our study concerning the role of two important adenosinetriphosphatases (ATPases) in a porcine model of spontaneous ICH that we have recent...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-013-9380-8
更新日期:2013-09-01 00:00:00
abstract::Allicin, one of the main biologically active compounds derived from garlic, was previously reported to possess multiple pharmacological activities. Whether allicin protected against chronic social defeat stress (CSDS) induced depressive-like behaviors remained unknown. Thus, our present study for the first time invest...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-018-0342-z
更新日期:2019-02-01 00:00:00
abstract::Hyperammonemia is necessary for development of the cerebral complications to liver disease including hepatic encephalopathy and cerebral edema but the mechanisms are unclear. Ammonia is taken up by the brain in proportion to its arterial concentration. The flux into the brain is most likely by both diffusion of NH3 an...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-014-9494-7
更新日期:2014-12-01 00:00:00
abstract::Several epidemiological studies have shown that Diabetes Mellitus (DM) or Insulin Resistance (IR) increases the risk of dementia. Besides, some authors suggested that poor glucose control to be associated with worse cognitive function. We aimed to assess cognitive functions and IR-degree over time in diabetic. We also...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-9977-4
更新日期:2017-06-01 00:00:00
abstract::We have described the occurrence in freely moving gerbils of slow potential changes (SPC) in two different models of experimental epilepsy: 1) maximal electroshock and 2) bilateral epileptic foci induced by penicillin. SPC is considered a by-product of epileptiform activity in both models and correlates to the SPC whi...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF01000149
更新日期:1992-06-01 00:00:00
abstract::The developing facial neurons of a series of hamsters ranging in age from the 14-day fetus to the 9 day postnatal were axotomized. Postoperative times were graded for each age so that the retrograde response could be observed before any significant amount of cell degeneration or death occurred. The incorporation of tr...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF00996894
更新日期:1993-06-01 00:00:00
abstract::Aberrant brain functional connectivity has been considered as the important mechanism underlying minimal hepatic encephalopathy (MHE); however, little is known about the change in interhemispheric connection in MHE patients. Twenty patients with HBV-related cirrhosis and MHE and 15 healthy controls were included in th...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9505-8
更新日期:2014-09-01 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::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: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::Mild hypothermia (32 degrees C-35 degrees C) reduces intracranial pressure in patients with acute liver failure and may offer an effective adjunct therapy in the management of these patients. Studies in experimental animals suggest that this beneficial effect of hypothermia is the result of a decrease in blood-brain a...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1023/a:1021982523691
更新日期:2002-12-01 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::Hepatic encephalopathy (HE) is an important cause of morbidity and mortality in patients with severe liver disease. Although the mechanisms responsible for HE remain elusive, ammonia is generally considered to be involved in its pathogenesis, and astrocytes are thought to be the principal target of ammonia neurotoxici...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1023/a:1023858902184
更新日期:2003-06-01 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
abstract::In view of the protective effect of brain derived neurotrophic factor (BDNF) against metabolic/excitotoxic insults in vitro, we investigated whether BDNF could limit infarct size after permanent occlusion of the middle cerebral artery in rat (MCAO). BDNF was delivered into the territory of the middle cerebral artery v...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF02674671
更新日期:1997-12-01 00:00:00
abstract::A high co-morbidity between Alzheimer's disease (AD) and depression suggests there might be similar mechanisms underlying the course of these diseases. Previous studies have shown that p38MAPK plays a critical role in the pathophysiology of AD and depression. However, little is known about whether SB203580, a selectiv...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9880-4
更新日期:2017-02-01 00:00:00
abstract::Severe neurological symptoms, cerebral edema, and atrophy are common features of the inherited metabolic disorder propionic acidemia. However, the pathomechanisms involved in the neuropathology of this disease are not well established. In this study, we investigate the effects of propionic acid (PA), a metabolite accu...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-006-9002-9
更新日期:2006-03-01 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:UNLABELLED:Some subjects with multiple sclerosis (MS) present with low blood iron parameters. Anecdotal reports and a single patient study suggest that iron supplementation may be beneficial in these subjects. Myelin is regenerated continually, but prerequisites for this process are iron and a functional folate-vitamin...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-006-9019-0
更新日期:2006-09-01 00:00:00
abstract::Type 2 diabetes (T2D) and Alzheimer's disease (AD) are two progressive disorders with high prevalence worldwide. Polymorphisms in tumor necrosis factor-alpha (TNF-α) and apolipoprotein E (ApoE) genes might be associated with both T2D and AD, representing possible genetic markers for the development of the AD in subjec...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-012-9310-1
更新日期:2012-12-01 00:00:00
abstract::Although gaseous ammonia (NH3) can freely enter cells through the plasma membrane where NH3 is cyto(neuro)toxic, NH3 and ionic ammonia (NH4+) contents have not been studied in biological materials. We developed a new method for measurement of expiratory NH3 concentration, which may reflect blood NH3 concentrations. Th...
journal_title:Metabolic brain disease
pub_type: 临床试验,杂志文章
doi:
更新日期:1997-06-01 00:00:00
abstract::Hepatic encephalopathy (HE) is a neuropsychiatric syndrome which develops in patients with severe liver diseases and/or portal-systemic shunting. Minimal HE, the earliest manifestation of HE, has drawn increasing attention in the last decade. Minimal HE is associated with a series of brain functional changes, such as ...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-014-9504-9
更新日期:2014-09-01 00:00:00
abstract::Glutaric aciduria type I (GA-I) is an inborn error of lysine and tryptophan metabolism. Clinical manifestations of GA-I include dystonic or dyskinetic cerebral palsy, but when the symptoms occur, treatment is not effective. In Taiwan, newborn screening for GA-I started in 2001; we wish to evaluate the outcomes of pati...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-012-9349-z
更新日期:2013-03-01 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::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