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 accumulating in this disorder, on cytoskeletal reorganization, on cell viability, and on the in vitro phosphorylation of glial fibrillary acidic protein (GFAP) and vimentin in cultured astrocytes from cerebral cortex of neonatal rats. We observed that the astrocytes changed their usual polygonal morphology when exposed to 5 mM PA for 72 h, leading to the appearance of fusiform or process-bearing cells, without elicit cell death. We also noticed that after 72 h treatment with 5 mM PA cells showed retracted cytoplasm with bipolar processes containing packed GFAP filaments and disorganized actin stress fibers, as revealed by immunocytochemistry. In addition, the morphological alterations were accompanied by increased in vitro 32P incorporation into GFAP and vimentin recovered into the high-salt Triton-insoluble cytoskeletal fraction. In conclusion, our results indicate that PA lead to cytoskeletal reorganization and to increased in vitro phosphorylation of Triton-insoluble GFAP and vimentin. On the basis of our results we could suppose that Triton-insoluble GFAP and vimentin hyperphosphorylation could be implicated in the reorganization of cellular structure and these findings could be involved in the brain damage characteristic of propionic acidemia patients.
journal_name
Metab Brain Disjournal_title
Metabolic brain diseaseauthors
de Almeida LM,Funchal C,Gottfried C,Wajner M,Pessoa-Pureur Rdoi
10.1007/s11011-006-9002-9subject
Has Abstractpub_date
2006-03-01 00:00:00pages
51-62issue
1eissn
0885-7490issn
1573-7365journal_volume
21pub_type
杂志文章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：:Alzheimer's disease (AD) is a progressive neurodegenerative disorder, characterized by accumulation and deposition of Aβ peptide in human brain. The present study aimed to determine the protective effect of catechin rich extract of MERM (methanolic extract of Rhizophora mucronata) on Aβ (25-35) induced cognitive impai...
journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-016-9831-0
更新日期：2016-08-01 00:00:00
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 be...
journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1023/b:mebr.0000027421.33085.8b
更新日期：2004-06-01 00:00:00
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：: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：: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
pub_type： 杂志文章,评审
doi：10.1007/BF02029492
更新日期：1996-12-01 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：: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：: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：:Antipsychotics, such as risperidone, increase food intake and induce alteration in glucose and lipid metabolism concomitantly with overweight and body fat increase, these biological abnormalities belong to the metabolic syndrome definition (high visceral adiposity, hypertriglyceridemia, hyperglycemia, low HDL-choleste...
journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-017-0133-y
更新日期：2018-02-01 00:00:00
abstract：:To assess whether EGb761 could protect elderly diabetic mice with cognitive disorders and explore the role of beclin-1-mediated autophagy in these protective effects. Two-month-old male db/db-/- mice and wild-type C57/BL6 mice were randomly divided into six groups: db/db-/- control, db/db-/- 50 mg, db/db-/- 100 mg, wi...
journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-018-0295-2
更新日期：2018-12-01 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：: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
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：: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：:Alcohol dependence (AD) has a large heritable component. Genetic variation in genes involved in the absorption and elimination of ethanol have been associated with AD. However, some of these polymorphisms are not present in an African population. Previous studies have reported that a type of AD which is characterized ...
journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-014-9503-x
更新日期：2014-06-01 00:00:00
abstract：:D-galactose (GAL) causes aging-related changes and oxidative stress in the organism. We investigated the effect of whole fresh blueberry (BB) (Vaccinium corymbosum L.) treatment on oxidative stress in age-related brain damage model. Rats received GAL (300 mg/kg; s.c.; 5 days per week) alone or together with 5 % (BB1) ...
journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-014-9643-z
更新日期：2015-06-01 00:00:00
abstract：:3-Nitropropionic acid (3-NPA) is a toxin sometimes produced on moldy crops (sugarcane, peanuts, etc.) in amounts sufficient to cause severe neurological disorders when consumed by humans. In vitro, 3-NPA irreversibly inactivates SDH, a Complex II respiratory enzyme required for mitochondrial energy production. A singl...
journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1023/a:1020753629477
更新日期：1999-06-01 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：:D-Galactose (D-gal) chronic administration via intraperitoneal and subcutaneous routes has been used as a model of aging and Alzheimer disease in rodents. Intraperitoneal and subcutaneous administration of D-gal causes memory impairments, a reduction in the neurogenesis of adult mice, an increase in the levels of the ...
journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1007/s11011-017-9972-9
更新日期：2017-06-01 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：:Depression is a complex progressive disorder accompanied by activation of inflammatory and Th-1 driven pathways, oxidative and nitrosative stress (O&NS), lowered antioxidant levels, mitochondrial dysfunctions, neuroprogression and increased bacterial translocation. In depression, activation of immuno-inflammatory path...
journal_title：Metabolic brain disease
pub_type： 杂志文章,meta分析,评审
doi：10.1007/s11011-012-9326-6
更新日期：2012-12-01 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：: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：: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
pub_type： 杂志文章
doi：10.1007/s11011-013-9466-3
更新日期：2014-03-01 00:00:00
abstract：:The regional energy status and the availability of metabolic substrates during brain development are important, since a variety of fetal metabolic insults have been increasingly implicated in the evolution of neonatal brain disorders. The response of the brain to a metabolic insult is determined, in large part, by the...
journal_title：Metabolic brain disease
pub_type： 杂志文章
doi：10.1023/a:1025503115837
更新日期：2003-09-01 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：: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：: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
pub_type： 杂志文章,评审
doi：10.1023/a:1020680624084
更新日期：1998-12-01 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
pub_type： 杂志文章,评审
doi：10.1007/s11011-020-00562-x
更新日期：2021-01-01 00:00:00
