Abstract:
:Niemann-Pick type C 1 (NPC1) disease is an autosomal recessive cholesterol transport defect resulting in a neurodegenerative process in patients mainly at an early age, although some patients may start with manifestation in adult. Since loss of myelin is considered as a main pathogenetic factor, the precise mechanism inducing dysmylination in NPC1 disease is still unclear. In the present study, a quantitative evaluation on the myelin protein and its regulatory factors of oligodendrocytes, such as SRY-related HMG-box 10 (Sox10), Yin Yang 1 factor (YY1) and myelin gene regulatory factor (MRF), in different parts of the brain and spinal cord was performed in NPC1-mutant mice. The results showed that NPC1 protein was expressed in oligodendrocytes and the amount of myelin protein was generally decreased in all parts of the brain and spinal cord in NPC1-mutant mice. Compared to wild type, the amount of Sox10 and YY1 was not different in NPC1-mutant mice, but MRF was significantly decreased, suggesting a possible mechanism perturbing differentiation of oligodendrocytes and the myelination process in the NPC1-mutant mouse.
journal_name
Metab Brain Disjournal_title
Metabolic brain diseaseauthors
Yan X,Lukas J,Witt M,Wree A,Hübner R,Frech M,Köhling R,Rolfs A,Luo Jdoi
10.1007/s11011-011-9263-9subject
Has Abstractpub_date
2011-12-01 00:00:00pages
299-306issue
4eissn
0885-7490issn
1573-7365journal_volume
26pub_type
杂志文章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::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::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::D-glycerate 2 kinase (DGK) is an enzyme that mediates the conversion of D-glycerate, an intermediate metabolite of serine and fructose metabolism, to 2-phosphoglycerate. Deficiency of DGK leads to accumulation of D-glycerate in various tissues and its massive excretion in urine. D-glyceric aciduria (DGA) is an autosom...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-019-0384-x
更新日期:2019-04-01 00:00:00
abstract::Cadmium is a toxic environmental and industrial pollutant. Cadmium toxicity has been reported to produce biochemical and behavioral dysfunction that may cause adverse effects on several organs including the central nervous system. The present study was designed to investigate the neurotoxic effects of Cadmium Chloride...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9578-4
更新日期:2015-02-01 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::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::Glutaric aciduria type 1 is a neurometabolic disorder, caused by riboflavin-dependent glutaryl-CoA dehydrogenase deficiency. As its consequence, accumulation of the putatively neurotoxic metabolites (glutaric and 3-hydroxyglutaric acids) in body tissues, but especially within the brain, is observed. Estimated incidenc...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-018-0357-5
更新日期:2019-04-01 00:00:00
abstract::Hepatic encephalopathy (HE) is a major complication of liver failure/disease which frequently develops during the progression of end-stage liver disease. This metabolic neuropsychiatric syndrome involves a spectrum of symptoms, including cognition impairment, attention deficits and motor dysfunction which eventually c...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-016-9843-9
更新日期:2016-12-01 00:00:00
abstract::Current data on the neurotrophic effects of steroid hormones suggest that, in brain and spinal cord regions containing receptor systems, steroids act at the level of RNA and protein synthesis to effect metabolic changes associated with nerve-cell survival, elaboration/maintenance of dendritic and axonal processes, syn...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/BF01001350
更新日期:1988-03-01 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::Soluble amyloid-β protein (Aβ) oligomers have been recognized to be early and key intermediates in Alzheimer's disease-related synaptic dysfunction. In this study, using in vitro electrophysiology, we investigated interactions of the acidic oligosaccharide sugar chain (AOSC), a marine-derived acidic oligosaccharide, w...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9521-8
更新日期:2014-09-01 00:00:00
abstract::Statins or HMG-CoA reductase inhibitors have been shown to be effective at lowering cholesterol levels, and the application of these molecules has gradually emerged as an attractive therapeutic strategy for neurodegenerative diseases. Epidemiological studies suggest that statin use is associated with a decreased incid...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0091-4
更新日期:2017-12-01 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::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::Patients with chronic liver disease manifest a high incidence (> 75%) of pallidal signal hyperintensity on T1-weighted Magnetic Resonance Imaging (MRI), the intensity of which correlates with blood manganese levels and the presence of extrapyramidal symptoms. A major cause of pallidal hyperintensity on T1-weighted MRI...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/BF02109357
更新日期:1995-12-01 00:00:00
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
更新日期:2006-09-01 00:00:00
abstract::Despite many clinical trials assessing the role of zinc in major depressive disorder (MDD), the conclusions still remain ambiguous. The aim of the present clinical study was to determine and comparison the zinc concentration in the blood of MDD patients (active stage or remission) and healthy volunteers (controls), as...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9888-9
更新日期:2017-02-01 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::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
pub_type: 杂志文章
doi:10.1007/s11011-019-00461-w
更新日期:2019-12-01 00:00:00
abstract::The associations of levels of apolipoprotein A1 (ApoA1) and apolipoprotein B and ApoB/A1 ratio and risk of a first stroke have not been reliably documented. We performed a meta-analysis to summarize the relationships and confirmed them in a case-control study. We identified relevant publications in PubMed and Embase d...
journal_title:Metabolic brain disease
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s11011-015-9732-7
更新日期:2015-12-01 00:00:00
abstract::The central nervous system is one of the most vulnerable organs affected by mercury toxicity. Both acute and chronic exposure to mercury is also known to cause a variety of neurological or psychiatric disorders. Here, the neuroprotective effect of berberine (BN; 100 mg/kg bwt) on mercuric chloride (HgCl2; 0.4 mg/kg bw...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-015-9652-6
更新日期:2015-08-01 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::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::In the current era, plants are frequently tested for its antidepressant potential. Therefore young coconut water, a commonly used plant based beverage, was selected to explore its antidepressant potential. Rodents were selected for this study and forced swim test was conducted to explore antidepressant activity. Analy...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9866-2
更新日期:2016-10-01 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::In the original publication of the article, author name Masoumeh Asadbegi was incorrectly written as Masoumeh Asadbeigi. The authors regret the oversight. ...
journal_title:Metabolic brain disease
pub_type: 杂志文章,已发布勘误
doi:10.1007/s11011-017-0114-1
更新日期:2017-12-01 00:00:00
abstract::The selective vulnerability of pyramidal neurons in the CA1 hippocampal region in ischemic rat brain may be preceded by regional alterations of energy metabolism during early reperfusion. We measured ATP, phosphocreatine (PCr), and glucose in paramedian and lateral CA1 and in an area showing little postischemic cell l...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF00999235
更新日期:1988-09-01 00:00:00
abstract::Substantial evidence demonstrated that maternal dietary nutrients can significantly determine the susceptibility to developing metabolic disorders in the offspring. Therefore, we aimed to investigate the later-life effects of maternal and postweaning diets interaction on epigenetic modification of the central nervous ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-015-9678-9
更新日期:2015-10-01 00:00:00
abstract::Mutations in the HTT gene, consisting of expansion of CAG triplets, cause the Huntington's disease (HD), one of the major neurodegenerative disorders. Formation of aggregates of mutant huntingtin (mHTT, the product of the mutant HTT gene) leads to cellular dysfunctions, and subsequent neurodegeneration which manifest ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-019-00405-4
更新日期:2019-06-01 00:00:00