Novel mutations and clinical outcomes of copper-histidine therapy in Menkes disease patients.

abstract：:Bilateral, symmetrical hyperintensity of the globus pallidus is observed in T1-weighted cerebral magnetic resonance images in from 52 to 100% of patients with chronic liver disease. No significant relationship exists between the presence of these cerebral changes in image signal intensity and the patients' neuropsychi...

journal_title：Metabolic brain disease

authors： Morgan MY

更新日期：1998-12-01 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

authors： Divakaran P,Pavlina T,Johnson RC,Cotter R,Madsen D,Wiggins R

更新日期：1986-06-01 00:00:00

abstract：:The impaired insulin signaling has been recognized as a common pathogenetic mechanism between diabetes and Alzheimer's disease (AD). In the progression of AD, brain is characterized by defective insulin receptor substrate-1 (IRS-1) and increased oxidative stress. Thymol, a monoterpene phenol isolated from medicinal he...

journal_title：Metabolic brain disease

authors： FangFang,Li H,Qin T,Li M,Ma S

更新日期：2017-04-01 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

authors： Narayanan SN,Kumar RS,Karun KM,Nayak SB,Bhat PG

更新日期：2015-10-01 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

authors： Haghighat N,McCandless DW,Geraminejad P

更新日期：1999-09-01 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

authors： Chastre A,Jiang W,Desjardins P,Butterworth RF

更新日期：2010-03-01 00:00:00

abstract：:Artificial sweeteners are mainly used as substitutes for sucrose derivates. In this study, we analyzed if the chronic consumption of aspartame or acesulfame potassium at an early age, produces histological alterations, astrogliosis and decreased neuronal viability, in hippocampus, prefrontal cortex, amygdala and hypot...

journal_title：Metabolic brain disease

authors： Solis-Medina A,Martínez-Magaña JJ,Quintanar-Jurado V,Gallegos-Silva I,Juárez-Rojop IE,Tovilla-Zárate CA,Díaz-Zagoya JC,Hernández-Díaz Y,González-Castro TB,López-Narváez ML,Genis-Mendoza AD,Nicolini H

更新日期：2018-12-01 00:00:00

abstract：:Stress-induced activation of the expression of the endoplasmic reticulum (ER)-resident chaperon and member of the protein disulfide isomerase family erp72 was studied after transient cerebral ischemia in vivo using the four-vessel occlusion method and experimental depletion of ER calcium stores in primary neuronal cel...

journal_title：Metabolic brain disease

authors： Paschen W,Gissel C,Linden T,Doutheil J

更新日期：1998-03-01 00:00:00

abstract：:Traumatic brain injury (TBI) results in severe neurological impairments without effective treatments. Inflammation appears to be an important contributor to key pathogenic events such as secondary brain injury following TBI and therefore serves as a promising target for novel therapies. We have recently demonstrated t...

journal_title：Metabolic brain disease

authors： Yang L,Liu Z,Ren H,Zhang L,Gao S,Ren L,Chai Z,Meza-Romero R,Benedek G,Vandenbark AA,Offner H,Li M

更新日期：2017-10-01 00:00:00

abstract：:In Alzheimer's disease (AD) basal forebrain cholinergic neurons appear to be targeted primarily in early stages of the disease. Scopolamine (muscarinic receptor antagonist) has been used for decades to induce working and reference memory impairment in rodents. In this study, we evaluated the protective effect of kolav...

journal_title：Metabolic brain disease

authors： Ishola IO,Adamson FM,Adeyemi OO

更新日期：2017-02-01 00:00:00

abstract：:Isolated Methylmalonic acidemia/aciduria (MMA) is a group of inborn errors of metabolism disease which is caused by defect in methylmalonyl-CoA mutase (MCM) enzyme. The enzyme has a key function in the catabolism of branched chain amino acids (BCAA, isoleucine, and valine), methionine, and threonine. MCM is encoded by...

journal_title：Metabolic brain disease

authors： Shafaat M,Alaee MR,Rahmanifar A,Setoodeh A,Razzaghy-Azar M,Bagherian H,Bagheri SD,Zafarghandi Motlagh F,Hashemi M,Abiri M,Zeinali S

更新日期：2018-10-01 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

authors： Li G,Ruan L,Chen R,Wang R,Xie X,Zhang M,Chen L,Yan Q,Reed M,Chen J,Xu Y,Pan J,Huang W

更新日期：2015-12-01 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

authors： Ghosh R,Sil S,Gupta P,Ghosh T

更新日期：2020-12-01 00:00:00

abstract：:As studies of brain metabolism grow in complexity, investigators turn increasingly to nuclear magnetic resonance spectroscopy combined with 13C isotopic labeling. The unique ability to detect labeling non-destructively in specific carbon positions of individual compounds has opened the way to investigate brain metabol...

journal_title：Metabolic brain disease

authors： Mason GF,Behar KL,Lai JC

更新日期：1996-12-01 00:00:00

abstract：:First-episode psychosis (FEP) patients are more sensitive to neuroleptic side-effects such as hyperprolactinemia. We examined the prolactin levels of previously minimally treated patients with first episode schizophrenia over their first year of treatment with flupenthixol decanoate and the relationship between prolac...

journal_title：Metabolic brain disease

authors： Retief M,Chiliza B,Phahladira L,Emsley R,Asmal L

更新日期：2019-12-01 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

authors： Clark P,Jones KJ,LaVelle A

更新日期：1993-06-01 00:00:00

abstract：:Due to the high ability of cadmium to cross the blood-brain barrier, cadmium (Cd) causes severe neurological damages. Hence, the purpose of this study was to investigate the possible protective effect of Mangifera indica leaf extract (MLE) against Cd-induced neurotoxicity. Rats were divided into eight groups. Group 1 ...

journal_title：Metabolic brain disease

authors： Al Omairi NE,Radwan OK,Alzahrani YA,Kassab RB

更新日期：2018-08-01 00:00:00

abstract：:This study presents further research into the spectrum of variants in genes responsible for the development of phenylketonuria (PKU) and hyperphenylalaninemia (HPA) in patients in Russia. After a study of 25 frequent variants, 293 patients (327 chromosomes without detected variants) from among 1265 probands still had ...

journal_title：Metabolic brain disease

authors： Kuznetcova I,Gundorova P,Ryzhkova O,Polyakov A

更新日期：2019-12-01 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

authors： Moseilhy A,Hassan MM,El Abd HS,Mohammad SA,El Bekay R,Abdel-Motal UM,Ouhtit A,Zaki OK,Zayed H

更新日期：2017-02-01 00:00:00

abstract：:Ischemic postconditioning (PostC) conventionally refers to a series of brief blood vessel occlusions and reperfusions, which can induce an endogenous neuroprotective effect and reduce cerebral ischemia/reperfusion (I/R) injury. Depending on the site of adaptive ischemic intervention, PostC can be classified as in situ...

journal_title：Metabolic brain disease

authors： Li CY,Ma W,Liu KP,Yang JW,Wang XB,Wu Z,Zhang T,Wang JW,Liu W,Liu J,Liang Y,Zhang XK,Li JJ,Guo JH,Li LY

更新日期：2021-01-01 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

authors： Moore ME,Koenig AE,Hillgartner MA,Otap CC,Barnby E,MacGregor GG

更新日期：2017-12-01 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

authors： Zhang LJ,Wu S,Ren J,Lu GM

更新日期：2014-09-01 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

authors： Gao W,Wang W,Liu G,Zhang J,Yang J,Deng Z

更新日期：2019-02-01 00:00:00

abstract：:Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system which causes chronic demyelination. Hydroxychloroquine (HCQ) possess immunosuppressive and anti-inflammatory properties. The aim of this study was to investigate the effect of HCQ on miR-219 and miR-155-3p expression changes in MS-...

journal_title：Metabolic brain disease

authors： Mazloumfard F,Mirian M,Eftekhari SM,Aliomrani M

更新日期：2020-12-01 00:00:00

abstract：:α-asarone (ASR) is a major bioactive compound isolated from the rhizome of Acorus tatarinowii Schott and it has extensive biological effects. Clinically, anxiety disorder is a common comorbidity of chronic pain. However, limited information is available regarding the effects of ASR on chronic pain-related anxiety. Thi...

journal_title：Metabolic brain disease

authors： Tian J,Tian Z,Qin SL,Zhao PY,Jiang X,Tian Z

更新日期：2017-12-01 00:00:00

abstract：:Iron is an important micronutrient for neuronal function and survival. It plays an essential role in DNA and protein synthesis, neurotransmission and electron transport chain due to its dual redox states. On the contrary, iron also catalyses the production of free radicals and hence, causes oxidative stress. Therefore...

journal_title：Metabolic brain disease

authors： Minhas G,Modgil S,Anand A

更新日期：2014-09-01 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

authors： Streck EL,Zugno AI,Tagliari B,Wannmacher C,Wajner M,Wyse AT

更新日期：2002-06-01 00:00:00

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 typic...

journal_title：Metabolic brain disease

authors： 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 EL

更新日期：2017-10-01 00:00:00

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

authors： 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 EL

更新日期：2020-01-01 00:00:00

abstract：:Pipecolic acid (PA) levels are increased in severe metabolic disorders of the central nervous system such as Zellweger syndrome, infantile Refsum disease, neonatal adrenoleukodystrophy and hyperlysinemia. The affected individuals present progressive neurological dysfunction, hypotonia and growth retardation. The mecha...

journal_title：Metabolic brain disease

authors： Dalazen GR,Terra M,Jacques CE,Coelho JG,Freitas R,Mazzola PN,Dutra-Filho CS

更新日期：2014-03-01 00:00:00