Complete Loss of Myelin protein zero (MPZ) in a patient with a late onset Charcot-Marie-Tooth (CMT)
AbstractCharcot-Marie-Tooth (CMT) comprises a group of hereditary neuropathies with clinical, epidemiological, and molecular heterogeneity in which variants in more than 80 different genes have been reported. One of the important genes which cause 5% of all CMT cases is Myelin protein zero (P0,MPZ). Variants in this gene have been reported in association with different forms of CMT including classical CMT1, severe DSS (CMT3B), DI-CMT, CMT2I and CMT2J with autosomal dominant (AD) inheritance. To our knowledge,MPZ variants have not been described in autosomal recessive (AR) form of CMT in previous studies. Moreover, its comp...
Source: Metabolic Brain Disease - March 23, 2023 Category: Neurology Source Type: research
Irisin attenuates ethanol-induced behavioral deficits in mice through activation of Nrf2 and inhibition of NF- κB pathways
This study aims to investigate the effect of irisin on ethanol-induced behavioral deficits and explore the underlying mechanisms. A mouse model of ethanol addiction/withdrawal was constructed through chronic ethanol administration. Depressive-like behaviors were evaluated by the tail suspension test and forced swimming test, and anxiety-like behaviors were evaluated by the marble-burying test and elevated plus maze test. The expression of Nrf2 was measured by western blotting. Levels of inflammatory mediators (NF- κB, TNF-α, IL-1β and IL-6) and oxidative stress factors (ROS, MDA, GSH and SOD) were detected by ELISA. The...
Source: Metabolic Brain Disease - March 22, 2023 Category: Neurology Source Type: research
Morus alba fruit diet ameliorates cognitive deficit in mouse model of streptozotocin-induced memory impairment
This study explored the preventive as well as therapeutic effects of diet enriched withMorus alba fruits extract (DEMA) in streptozotocin (STZ) induced mouse model of memory impairment. The study consisted of two facets: one aspect consisted of pretreatment of animals with DEMA for two weeks followed by STZ (i.c.v) intervention and the second phase involved induction of dementia with STZ (i.c.v) followed by treatment with DEMA for 14 days. Cognitive functions of animals were measured by Morris Water Maze test and to delineate the associated mechanism of action, brain biochemical estimations (acetyl-cholinesterase activit...
Source: Metabolic Brain Disease - March 22, 2023 Category: Neurology Source Type: research
Irisin attenuates ethanol-induced behavioral deficits in mice through activation of Nrf2 and inhibition of NF- κB pathways
This study aims to investigate the effect of irisin on ethanol-induced behavioral deficits and explore the underlying mechanisms. A mouse model of ethanol addiction/withdrawal was constructed through chronic ethanol administration. Depressive-like behaviors were evaluated by the tail suspension test and forced swimming test, and anxiety-like behaviors were evaluated by the marble-burying test and elevated plus maze test. The expression of Nrf2 was measured by western blotting. Levels of inflammatory mediators (NF- κB, TNF-α, IL-1β and IL-6) and oxidative stress factors (ROS, MDA, GSH and SOD) were detected by ELISA. The...
Source: Metabolic Brain Disease - March 22, 2023 Category: Neurology Source Type: research
Morus alba fruit diet ameliorates cognitive deficit in mouse model of streptozotocin-induced memory impairment
This study explored the preventive as well as therapeutic effects of diet enriched withMorus alba fruits extract (DEMA) in streptozotocin (STZ) induced mouse model of memory impairment. The study consisted of two facets: one aspect consisted of pretreatment of animals with DEMA for two weeks followed by STZ (i.c.v) intervention and the second phase involved induction of dementia with STZ (i.c.v) followed by treatment with DEMA for 14 days. Cognitive functions of animals were measured by Morris Water Maze test and to delineate the associated mechanism of action, brain biochemical estimations (acetyl-cholinesterase activit...
Source: Metabolic Brain Disease - March 22, 2023 Category: Neurology Source Type: research
Stellate ganglion block suppresses hippocampal ferroptosis to attenuate cerebral ischemia-reperfusion injury via the Hippo pathway
In conclusion, SGB inhibited ferroptosis of hippocampal neurons via activating the Hippo pathway and thereby alleviated I/R injury. The data provide a novel insight into the treatment of ischemic stroke and even other ischemic encephalopathies.Graphical Abstract (Source: Metabolic Brain Disease)
Source: Metabolic Brain Disease - March 16, 2023 Category: Neurology Source Type: research
Hsa_circ_0001361 facilitates cell progression and glycolytic metabolism in neuroblastoma via interacting with mir-490-5p to induce TRIM2 upregulation
This study aimed to investigate the functional mechanism of hsa_circ_0001361 in NB. Hsa_circ_0001361, miR-490-5p and tripartite motif 2 (TRIM2) were detected through reverse transcription-quantitative polymerase chain reaction. The proliferation ability was examined using cell counting kit-8 assay, colony formation assay and ethynyl-2’-deoxyuridine assay. Cell migration and inv asion were assessed via transwell assay and wound healing assay. The protein levels were measured by western blot. Glycolysis was analyzed via commercial kits. Dual-luciferase reporter assay and RNA immunoprecipitation assay were performed for t...
Source: Metabolic Brain Disease - March 15, 2023 Category: Neurology Source Type: research
Ferrous sulfate reverses cerebral metabolic abnormality induced by minimal hepatic encephalopathy
In this study, MHE was induced in rats by partial portal vein ligation (PPVL), and was treated with ferrous sulfate. The Morris water maze was used to evaluate the cognitive condition of the rats. The metabolites observed by NMR and validated by liquid chromatography –mass spectrometry were defined as the key affected metabolites. The enzyme activities and trace element contents in the rat brains were also investigated. The Mn content was found to be increased but the ferrous iron content decreased in the cortex and striatum in MHE. Decreased oxoglutarate dehy drogenase activity and increased glutamine synthetase (GS) an...
Source: Metabolic Brain Disease - March 14, 2023 Category: Neurology Source Type: research
Circular RNA circ_0000741/miR-379-5p/TRIM14 signaling axis promotes HDAC inhibitor (SAHA) tolerance in glioblastoma
ConclusionCirc_0000741 might accelerate SAHA tolerance by regulating the miR-379-5p/TRIM14 axis, which provided a promising therapeutic target for GBM treatment. (Source: Metabolic Brain Disease)
Source: Metabolic Brain Disease - March 11, 2023 Category: Neurology Source Type: research
