Abstract:
:Tissue methylmalonic acid (MMA) accumulation is the biochemical hallmark of methylmalonic acidemia. Clinically, the disease is characterized by progressive neurological deterioration and renal failure, whose pathophysiology is still undefined. In the present study we investigated the effect of acute MMA administration on some important parameters of brain neurotransmission in cerebral cortex of rats, namely Na(+), K(+)-ATPase, ouabain-insensitive ATPases and acetylcholinesterase activities, in the presence or absence of kidney injury induced by gentamicin administration. Initially, thirty-day old Wistar rats received one intraperitoneal injection of saline or gentamicin (70 mg/kg). One hour after, the animals received three consecutive subcutaneous injections of MMA (1.67 μmol/g) or saline, with an 11 h interval between each injection. One hour after the last injection the animals were killed and the cerebral cortex isolated. MMA administration by itself was not able to modify Na(+), K(+)-ATPase, ATPases ouabain-insensitive or acetylcholinesterase activities in cerebral cortex of young rats. In rats receiving gentamicin simultaneously with MMA, it was observed an increase in the activity of acetylcholinesterase activity in cerebral cortex, without any alteration in the activity of the other studied enzymes. Therefore, it may be speculated that cholinergic imbalance may play a role in the pathogenesis of the brain damage. Furthermore, the pathophysiology of tissue damage cannot be exclusively attributed to MMA toxicity, and control of kidney function should be considered as a priority in the management of these patients, specifically during episodes of metabolic decompensation when MMA levels are higher.
journal_name
Metab Brain Disjournal_title
Metabolic brain diseaseauthors
Affonso AC,Machado DG,Malgarin F,Fraga DB,Ghedim F,Zugno A,Streck EL,Schuck PF,Ferreira GCdoi
10.1007/s11011-013-9396-0subject
Has Abstractpub_date
2013-09-01 00:00:00pages
493-500issue
3eissn
0885-7490issn
1573-7365journal_volume
28pub_type
杂志文章abstract::The experimental model of central pontine myelinolysis--chronic (4-day) hyponatremia induced by daily injections of hypotonic dextrose solutions and vasopressin followed by rapid correction with saline--was used in young fasted and thirsted mice. In normal controls chronic fasting and thirsting lowered plasma and brai...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF00999694
更新日期:1987-12-01 00:00:00
abstract::Increased benzodiazepine (BZ) receptor density has been reported in brains of rabbits with hepatic encephalopathy (HE) due to galactosamine (GalN)-induced fulminant hepatic failure (FHF). These data were generated using detergent-Triton X-100-treated neural membranes. While performing further studies it was noted that...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF01000296
更新日期:1989-09-01 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::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::While risk factors for konzo are known, determinants of cognitive impairment in konzo-affected children remain unknown. We anchored cognitive performance (KABC-II scores) to serum levels of free-thyroxine (free-T4), thyroid-stimulating hormone (TSH), albumin, and motor proficiency (BOT-2 scores) in 40 children includi...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9492-9
更新日期:2014-06-01 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
pub_type: 杂志文章
doi:10.1007/s11011-019-00474-5
更新日期:2019-12-01 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::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::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
pub_type: 杂志文章
doi:10.1007/s11011-010-9185-y
更新日期:2010-03-01 00:00:00
abstract::To investigate the short-term brain activity changes in cirrhotic patients with Liver transplantation (LT) using resting-state functional MRI (fMRI) with regional homogeneity (ReHo) method. Twenty-six cirrhotic patients as transplant candidates and 26 healthy controls were included in this study. The assessment was re...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-015-9657-1
更新日期:2015-08-01 00:00:00
abstract::We examined how serum cholesterol, an established risk factor for cerebrovascular disease (CVD), relates to cognitive function in healthy middle-older aged individuals with no neurologic or CVD history. A complete lipid panel was obtained from a cohort of one hundred twenty individuals, ages 43-85, who also underwent ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9797-y
更新日期:2016-04-01 00:00:00
abstract::The associations between thyroid auto-immunity and neuro-psychiatric disorders are well-documented. However, there exists limited literature specifically linking auto-immune thyroid disease (AITD) to bipolar disorder (BD). Thus, we investigated the likely association between Hashimoto's disease and BD through the extr...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-019-00437-w
更新日期:2019-10-01 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::Cyclic AMP is part of an endogenous mechanism that downregulates inflammatory response, and its intracellular concentration is regulated chiefly by cyclic nucleotide phosphodiesterases type 4. The goal of the present study was to determine whether phosphodiesterases 4 are involved in the inflammatory response of astro...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-009-9150-9
更新日期:2009-09-01 00:00:00
abstract::Studies have shown that oxidative stress is involved in the pathophysiology of bipolar disorder (BD). It is suggested that omega-3 (ω3) fatty acids are fundamental to maintaining the functional integrity of the central nervous system. The animal model used in this study displayed fenproporex-induced hyperactivity, a s...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9942-7
更新日期:2017-04-01 00:00:00
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
doi:10.1023/a:1021941414605
更新日期:2002-12-01 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
pub_type: 杂志文章
doi:10.1007/BF00999385
更新日期:1986-06-01 00:00:00
abstract::Minimal hepatic encephalopathy (MHE) is the earliest form of hepatic encephalopathy (HE) and affects up to 80 % of patients with liver cirrhosis. By definition, MHE is characterized by psychomotor slowing and subtle cognitive deficits, but obvious clinical manifestations are lacking. Given its covert nature, MHE is o...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9848-4
更新日期:2016-10-01 00:00:00
abstract::There is substantial clinical and experimental evidence that ammonia is a major factor in the pathogenesis of hepatic encephalopathy. In the article is demonstrated that in hepatocellular dysfunction, ammonia detoxification to glutamine (GLN) in skeletal muscle, brain, and likely the lungs, is activated. In addition t...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-013-9428-9
更新日期:2014-03-01 00:00:00
abstract::Chronic liver disease (CLD) leads to a spectrum of neuropsychiatric disorders named hepatic encephalopathy (HE). Even though brain energy metabolism is believed to be altered in chronic HE, few studies have explored energy metabolism in CLD-induced HE, and their findings were inconsistent. The aim of this study was to...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-015-9715-8
更新日期:2016-12-01 00:00:00
abstract::Increasing evidence indicates that fetal metabolic stress may result in a variety of post-natal perturbations during brain development. The goal of the study was to determine the duration of hypoxia/ischemia that would elicit a demonstrable regional depression of metabolism in the fetal brain and further to examine se...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-006-9031-4
更新日期:2006-12-01 00:00:00
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
doi:10.1007/s11011-012-9342-6
更新日期:2013-03-01 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::The heterogeneity of monoamine oxidase (MAO; EC 1.4.3.4) activities was studied in two fractions of synaptic mitochondria (SM & SM2) and one fraction of non-synaptic ("free") mitochondria (M) isolated from three rat brain regions (cerebral cortex, striatum, and pons & medulla) by the Lai and Clark (1979, 1989) method ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF01996074
更新日期:1994-03-01 00:00:00
abstract::Near infrared radiation (NIR) is known to penetrate and affect biological systems in multiple ways. Recently, a series of experimental studies suggested that low intensity NIR may protect neuronal cells against a wide range of insults that mimic diseases such as stroke, brain trauma and neurodegeneration. However, the...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9515-6
更新日期:2015-04-01 00:00:00
abstract::Prenatal stress has been associated with increased vulnerability to psychiatric disturbances including schizophrenia, depression, attention-deficit hyperactivity disorder and autism. Elevated maternal circulating stress hormones alter development of neural circuits in the fetal brain and cause long-term changes in beh...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-009-9161-6
更新日期:2009-12-01 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::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
pub_type: 杂志文章
doi:10.1007/s11011-020-00609-z
更新日期:2020-12-01 00:00:00
abstract::High plasma levels of methionine (Met) and its metabolites such as methionine sulfoxide (MetO) may occur in several genetic abnormalities. Patients with hypermethioninemia can present neurological dysfunction; however, the neurotoxicity mechanisms induced by these amino acids remain unknown. The aim of the present wor...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0054-9
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
pub_type: 杂志文章
doi:10.1007/s11011-019-00511-3
更新日期:2020-01-01 00:00:00