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Metabolic Brain Disease

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https://www.readbyqxmd.com/read/30244301/mednik-syndrome-with-a-frame-shift-causing-mutation-in-ap1s1-gene-and-literature-review-of-the-clinical-features
#1
Faruk Incecik, Atil Bisgin, Mustafa Yılmaz
MEDNIK syndrome is an autosomal recessive rare disease as one of the most recently described copper metabolism disorder characterized by intellectual disability, ichthyosis, hearing loss, peripheral neuropathy, enteropathy and keratodermia. Here in, we reported a case presented with ichthyosis and intellectual disability with MEDNIK syndrome that confirmed by mutation analysis in a Turkish child. She was finally diagnosed with MEDNIK syndrome by clinical findings, which were confirmed by molecular genetic testing...
September 23, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30242734/the-metabolic-alterations-within-the-normal-appearing-brain-in-patients-with-hashimoto-s-thyroiditis-are-correlated-with-hormonal-changes
#2
Joanna Bladowska, Marta Waliszewska-Prosół, Maria Ejma, Marek Sąsiadek
Hashimoto's thyroiditis (HT) is the most common autoimmune disease in humans usually associated with subsequent hypothyroidism. The purpose of the study was to assess metabolic alterations within the normal appearing brain in subjects with HT using MR spectroscopy (MRS) and to correlate MRS measurements with hormonal concentrations. Fifty-five HT patients (mean age 43.5 yrs) and 30 healthy controls (mean age 42.5 yrs) were examined with the use of a 1.5 T MR scanner. There were no signs of central nervous system involvement in the studied group...
September 21, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30229386/carvacrol-mitigates-proconvulsive-effects-of-lipopolysaccharide-possibly-through-the-hippocampal-cyclooxygenase-2-inhibition
#3
Mehdi Sadegh, Mohammad Hassan Sakhaie
Systemic injection of LPS changes neuronal excitability and increase susceptibility for convulsions. Carvacrol exerts neuroprotective and antiepileptic effects in animal models. Herein, we investigated the anticonvulsive effect of carvacrol on LPS induced seizure severity and possible involvement of the hippocampal COX-1 and -2 activities in this effect. Adult male wistar rats were used. LPS was injected (400 μg/kg; i.p.) four hours before the PTZ (80 mg/kg; i.p.) injection. Carvacrol was injected (100 mg/kg; i...
September 18, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30229385/standardized-citrus-unshiu-peel-extract-ameliorates-dexamethasone-induced-neurotoxicity-and-depressive-like-behaviors-in-mice
#4
Dong Wook Lim, Min Young Um, Taewon Han, Jaekwang Lee, Yun Tai Kim, Suengmok Cho, In-Ho Kim, Daeseok Han, Changho Lee
Dried Citrus unshiu peel, also known as Chinpi, have been commonly used as a traditional medicine to improve for allergy, inflammation and hepatopathy. Many previously studies have reported that citrus flavonoids show neuroprotective activities. However, the antidepressant-related effects of C. unshiu peels have not been well characterized. Here, the antidepressant-like effects of standardized C. unshiu peel extract (SCP) were evaluated in in vivo and in vitro depression models induced by dexamethasone (DEX), a synthetic glucocorticoid...
September 18, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30229384/leptin-stimulates-the-release-of-pro-inflammatory-cytokines-in-hypothalamic-astrocyte-cultures-from-adult-and-aged-rats
#5
Camila Leite Santos, Larissa Daniele Bobermin, Diogo Onofre Souza, André Quincozes-Santos
Leptin is an adipose tissue-derived hormone that acts on the hypothalamus in order to maintain energy homeostasis. However, leptin can also induce an inflammatory response. Increasing evidence has highlighted a critical role of astrocytes in the effects of leptin on the hypothalamus. In addition, astrocytes participate in neuroinflammation by producing and releasing a wide range of inflammatory mediators. In this study, we aimed to investigate the age-dependent effect of leptin on pro- and anti-inflammatory cytokines released by the hypothalamic astrocyte cultures obtained from newborn, adult, and aged Wistar rats...
September 18, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30220073/quality-of-life-related-to-clinical-features-in-patients-with-rett-syndrome-and-their-parents-a-systematic-review
#6
REVIEW
Silvia Corchón, Irene Carrillo-López, Omar Cauli
Patients with a neurodevelopmental disorder such as Rett syndrome (RS), as well as their families, have complex needs that affect their quality of life (QoL). Therefore, both families and patients with RS must be provided with multidisciplinary health care that can identify the clinical features that most affect their QoL and mental health risks. The main objective of this paper is to provide a comprehensive overview of the QoL subdimensions of families affected by RS, including both the parents and children...
September 16, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30218440/the-association-of-sleep-disordered-breathing-and-white-matter-hyperintensities-in-heart-failure-patients
#7
Chooza Moon, Barbara B Bendlin, Kelsey E Melah, Lisa C Bratzke
Heart failure patients often manifest white matter hyperintensites on brain magnetic resonance imaging (MRI). White matter hyperintnsities have also been linked with cognitive problems in patients with heart failure. Sleep disordered breathing may contribute to structural brain changes in heart failure. The purpose of this study was to test the extent to which the apnea hypopnea index is associated with global and regional white matter hyperintensities, and is a moderating factor in the relationship between age and white matter hyperintensites...
September 14, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30203378/changes-in-biophysical-characteristics-of-pfn1-due-to-mutation-causing-amyotrophic-lateral-sclerosis
#8
Mina Nekouei, Parviz Ghezellou, Atousa Aliahmadi, Sareh Arjmand, Mahmoud Kiaei, Alireza Ghassempour
Single amino acid mutations in profilin 1 (PFN1) have been found to cause amyotrophic lateral sclerosis (ALS). Recently, we developed a mouse model for ALS using a PFN1 mutation (glycine 118 to valine, G118V), and we are now interested in understanding how PFN1 becomes toxically lethal with only one amino acid substitution. Therefore, we studied mutation-related changes in the PFN1 protein and hypothesized that such changes significantly disturb its structure. Initially, we expressed and studied the purified PFN1WT and PFN1G118V proteins from bacterial culture...
September 10, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30187180/egb761-improves-the-cognitive-function-of-elderly-db-db-diabetic-mice-by-regulating-the-beclin-1-and-nf-%C3%AE%C2%BAb-signaling-pathways
#9
Zhu-Fei Guan, Xiao-Ming Zhang, Ying-Hong Tao, Yu Zhang, Yan-Yan Huang, Gang Chen, Wei-Jun Tang, Gang Ji, Qi-Lin Guo, Ming Liu, Qian Zhang, Na-Na Wang, Zhong-Yu Yu, Hao-Yang, Guo-Feng Wu, Zhou-Ping Tang, Zun-Guo Du, Xi-Liang Shang, Ying-Chao Liu, Guang-Hai Mei, Jing-Chun Guo, Hou-Guang Zhou
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, wild-type (WT) control, WT 50 mg, and WT 100 mg. EGb761 (50 mg/kg or 100 mg/kg of bodyweight) was given by gavage once a day for 1 month from the age of 6 months. Y-maze and social choice tests were performed at 8th months...
September 5, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30178281/contourlet-based-hippocampal-magnetic-resonance-imaging-texture-features-for-multivariant-classification-and-prediction-of-alzheimer-s-disease
#10
Ni Gao, Li-Xin Tao, Jian Huang, Feng Zhang, Xia Li, Finbarr O'Sullivan, Si-Peng Chen, Si-Jia Tian, Gehendra Mahara, Yan-Xia Luo, Qi Gao, Xiang-Tong Liu, Wei Wang, Zhi-Gang Liang, Xiu-Hua Guo
The study is aimed to assess whether the addition of contourlet-based hippocampal magnetic resonance imaging (MRI) texture features to multivariant models improves the classification of Alzheimer's disease (AD) and the prediction of mild cognitive impairment (MCI) conversion, and to evaluate whether Gaussian process (GP) and partial least squares (PLS) are feasible in developing multivariant models in this context. Clinical and MRI data of 58 patients with probable AD, 147 with MCI, and 94 normal controls (NCs) were collected...
September 3, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30171513/correction-to-therapeutic-effects-of-probiotics-on-neurotoxicity-induced-by-clindamycin-and-propionic-acid-in-juvenile-hamsters
#11
Nora Al-Orf, Afaf El-Ansary, Geir Bjørklund, Nadine Moubayed, Ramesa Shafi Bhat, Abir Ben Bacha
The original version of this article unfortunately contained a mistake. The family name of the fourth author listed in the title was incorrect, and the correct name is Nadine Moubayed, as noted in the addresses. Her name is now corrected in the author group of this article.
August 31, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30136092/antioxidant-and-antidepressant-like-effects-of-eugenia-catharinensis-d-legrand-in-an-animal-model-of-depression-induced-by-corticosterone
#12
Sara Cristiane Barauna, Débora Delwing-Dal Magro, Maitê Beatriz Brueckheimer, Thayná P Maia, Geraldo Antonio Bunick Neto Sala, André Wolff Döhler, Mateus Campestrini Harger, Dayse Fabiane Machado de Melo, André Luís de Gasper, Michele Debiasi Alberton, Diogo Alexandre Siebert, Gustavo Amadeu Micke, Cláudia Almeida Coelho de Albuquerque, Daniela Delwing-De Lima
This work investigated the antioxidant and antidepressant-like effects of ethyl acetate extract from Eugenia catharinensis in mice treated with corticosterone (20 mg/Kg). The animals received saline or corticosterone (21 days) and, in the last 7 days, they were treated with the extract (50, 125, 200 or 250 mg/Kg) or vehicle. After 24 h, the mice were submitted to the open field and forced swimming tests, after which the hippocampus and cerebral cortex were removed. Our results showed that the extract decreased the immobility time of mice in the forced swimming test and that the extract was able to reverse the effect caused by corticosterone...
August 22, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30120672/mitochondrial-alterations-accompanied-by-oxidative-stress-conditions-in-skin-fibroblasts-of-huntington-s-disease-patients
#13
Paulina Jędrak, Paweł Mozolewski, Grzegorz Węgrzyn, Mariusz R Więckowski
Huntington disease (HD) is an autosomal dominant neurodegenerative disorder manifesting as progressive impairment of motor function and different neuropsychiatric symptoms caused by an expansion of CAG repeats in huntingtin gene (HTT). Mitochondrial dysfunction and bioenergetic defects can contribute to the course of the disease, however, the molecular mechanism underlying this process is still largely unknown. In this study, we aimed to determine several mitochondrial parameters in HD fibroblasts and assess their relevance to the disease progression as well as to value mitochondrial pathology in peripheral cells as disease potential biomarker...
August 17, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30117100/l-3-n-butylphthalide-reduces-ischemic-stroke-injury-and-increases-m2-microglial-polarization
#14
Fangfang Li, Qingfeng Ma, Haiping Zhao, Rongliang Wang, Zhen Tao, Zhibin Fan, Sijia Zhang, Guangwen Li, Yumin Luo
Overwhelming evidence suggests that microglia play an important role in ischemic injury and they polarize into two different phenotypes with distinct functions after ischemic stroke. We performed the present study to investigate whether L-3-n butylphthalide (NBP) has an effect on microglial polarization. Mice were subjected to transient middle cerebral artery occlusion (MCAO) for 45 min, and then immediately after reperfusion were treated with NBP or vehicle via the caudal vein for 7 consecutive days. 2,3,5-Triphenyltetrazolium chloride (TTC) staining showed that NBP treatment resulted in a tendency to decrease cerebral infarct volume at 1 day after MCAO, and significant decreased infarct volume at 3 days after MCAO...
August 16, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30105614/donepezil-improves-the-cognitive-impairment-in-a-tree-shrew-model-of-alzheimer-s-disease-induced-by-amyloid-%C3%AE-1-40-via-activating-the-bdnf-trkb-signal-pathway
#15
Hong Zheng, Shiwei Niu, Hongbin Zhao, Shude Li, Jianlin Jiao
Alzheimer's disease (AD) is a chronic neurodegenerative disorder which can contribute to memory loss and cognitive damage in the elderly; moreover, evidence from clinical and animal studies demonstrated that AD always exhibit severe cognitive deficits. However, the effects of donepezil medications on cognition are controversial. Additionally, it is unclear whether donepezil can protect neurons to improve cognitive function through the brain-derived neurotropic factor (BDNF)/tyrosine receptor kinase B (TrkB) signalling pathway in the tree shrew (TS), which has a closer evolutionary relationship to primates than rodents...
August 13, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30105613/correlation-of-serum-levels-and-gene-expression-of-tumor-necrosis-factor-%C3%AE-induced-protein-8-like-2-with-parkinson-disease-severity
#16
Ebrahim Kouchaki, Reza Daneshvar Kakhaki, Omid Reza Tamtaji, Ehsan Dadgostar, Mohammad Behnam, Alireza Zaribaf, Hassan Nikoueinejad, Hossein Akbari, Zatollah Asemi
Different immune-mediated mechanisms involved in the pathogenesis of Parkinson disease (PD) as a neurodegenerative and inflammatory disease. According to our knowledge, there is no report evaluating Tumor necrosis factor-α-induced protein-8 like-2 (TIPE2), a cytokine maintaining immune homeostasis, in PD. We analyzed the correlation of the serum levels and circulatory gene expression of TIPE2 with severity of PD. In this case-control study, 43 patients with PD and 40 healthy subjects were enrolled. The diagnosis of PD was performed byclinical diagnostic criteria of the UK Parkinson's Disease Society Brain Bank...
August 13, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30094804/high-methionine-low-folate-and-low-vitamin-b6-b12-hm-lf-lv-diet-causes-neurodegeneration-and-subsequent-short-term-memory-loss
#17
Mohammed Nuru, Nino Muradashvili, Anuradha Kalani, David Lominadze, Neetu Tyagi
Methionine is an essential amino acid found in rich quantities in average American diet such as meats, fish and eggs. Excessive consumption of such food often exceeds the normal requirement of the methionine in our body; which found to be related to the development of neurodegenerative disorders. However, the mechanistic pathways of methionine's influence on the brain are unclear. The present study is focus on the effects of high methionine, low folate and low vitamin B6/B12 (HM-LF-LV) diet on the dysfunction of neuronal and vascular specific markers in the brain...
August 9, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30090953/ameliorative-effects-of-hydrogen-sulfide-nahs-on-chronic-kidney-disease-induced-brain-dysfunction-in-rats-implication-on-role-of-nitric-oxide-no-signaling
#18
Hassan Askari, Mohammad Foad Abazari, Pegah Ghoraeian, Sepehr Torabinejad, Maryam Nouri Aleagha, Reza Mirfallah Nassiri, Farshid Tahmasebi, Nairi Abedi, Sulail Fatima Rajani, Ali Salarian, Maryam Belaran, Mohammed Elshiekh, Nima Sanadgol
Chronic kidney disease (CKD) is a major public health problem worldwide and is associated with spatial learning deficits. The aim of the present study was to evaluate the protective effects of hydrogen sulfide (H2S) on CKD-mediated behavioral deficits with emphasis to the role of nitric oxide (NO) in these effects. Fifty rats were randomly allocated to five experimental groups including: sham, Five-sixth (5/6) nephrectomy (Nx), 5/6Nx + NaHS, 5/6Nx + NaHS+L-nitroarginine methyl ester (L-NAME), and 5/6Nx + NaHS+aminoguanidine (AMG)...
August 8, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30069621/alteration-in-cognitive-behaviour-brain-antioxidant-enzyme-activity-and-their-gene-expression-in-f1-generation-mice-following-cd-exposure-during-the-late-gestation-period-modulation-by-quercetin
#19
Sumita Halder, Rajarshi Kar, Nilesh Chandra, Archana Nimesh, Ashish K Mehta, Swapan K Bhattacharya, Pramod K Mediratta, Basu D Banerjee
We investigated whether in-utero Cd(II) chloride exposure of the dams between 14th to 21st day of gestation affects memory and learning, oxidative stress, antioxidant enzyme activity and their gene expression in brain of the pups in their adulthood. In the Morris water maze, cadmium (Cd) exposure impaired spatial memory which was reversed following co-treatment with quercetin (100 mg/kg). In the passive avoidance paradigm, retention memory was adversely affected but was significantly reversed by co treatment with quercetin (25, 50, 100 mg/kg)...
August 1, 2018: Metabolic Brain Disease
https://www.readbyqxmd.com/read/30066125/reply-to-contribution-of-the-mrps22-variant-and-a-down-mosaic-to-the-phenotype
#20
LETTER
Mustafa Kılıç, Esra Kılıç, Didem Yücel Yılmaz, Rıza Köksal Özgül
No abstract text is available yet for this article.
July 31, 2018: Metabolic Brain Disease
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