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Shin-ichi Tokushige Tomohiro Sonoo Risa Maekawa Yuichiro Shirota Ritsuko Hanajima Yasuo Terao Hideyuki Matsumoto Mohammad Arif Hossain Norio Sakai Yasushi Shiio 《Brain & development》2013
This report describes a 60-year-old female patient with Krabbe disease who presented with slowly progressive gait disturbance due to mild spastic paraplegia. Brain magnetic resonance imaging showed high-intensity lesions along the upper parts of the bilateral pyramidal tracts in fluid-attenuated inversion recovery images. Central motor conduction time was prolonged both in the upper and the lower extremities, while central sensory conduction time was normal. The reduced lymphocyte galactocerebrosidase (GALC) activity and two novel mutations in the GALC gene, p.G496S and p.G569S, proved the diagnosis of Krabbe disease. Our findings show that adult-onset Krabbe disease is characterized by isolated pyramidal tract impairment in the central nervous system, both neurophysiologically and radiologically. 相似文献
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Anna Blasiak Tomasz Blasiak Marian H. Lewandowski Mohammed Akhter Hossain John D. Wade Andrew L. Gundlach 《The European journal of neuroscience》2013,37(8):1284-1294
Behavioural state is controlled by a range of neural systems that are sensitive to internal and external stimuli. The relaxin‐3 and relaxin family peptide receptor 3 (RXFP3) system has emerged as a putative ascending arousal network with putative involvement in regulation of stress responses, neuroendocrine control, feeding and metabolism, circadian activity and cognition. Relaxin‐3/γ‐aminobutyric acid neuron populations have been identified in the nucleus incertus, pontine raphe nucleus, periaqueductal grey (PAG) and an area dorsal to the substantia nigra. Relaxin‐3‐positive fibres/terminals densely innervate arousal‐related structures in the brainstem, hypothalamus and limbic forebrain, but the functional significance of the heterogeneous relaxin‐3 neuron distribution and its inputs to specific brain areas are unclear. Therefore, in this study, we used neuronal tract‐tracing and immunofluorescence staining to explore the source of the dense relaxin‐3 innervation of the intergeniculate leaflet (IGL) of the thalamus, a component of the neural circadian timing system. Confocal microscopy analysis revealed that relaxin‐3‐positive neurons retrogradely labelled from the IGL were predominantly present in the PAG and these neurons expressed corticotropin‐releasing factor receptor‐like immunoreactivity. Subsequently, whole‐cell patch‐clamp recordings revealed heterogeneous effects of RXFP3 activation in the IGL by the RXFP3 agonist, relaxin‐3 B‐chain/insulin‐like peptide‐5 A‐chain (R3/I5). Identified, neuropeptide Y‐positive IGL neurons, known to influence suprachiasmatic nucleus activity, were excited by R3/I5, whereas neurons of unidentified neurotransmitter content were either depolarized or displayed a decrease in action potential firing and/or membrane potential hyperpolarization. Our data identify a PAG to IGL relaxin‐3/RXFP3 pathway that might convey stress‐related information to key elements of the circadian system and influence behavioural state rhythmicity. 相似文献
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Satyaveni Malasala Jitendra Gour Md. Naiyaz Ahmad Srikanth Gatadi Manjulika Shukla Grace Kaul Arunava Dasgupta Y. V. Madhavi Sidharth Chopra Srinivas Nanduri 《RSC advances》2020,10(71):43533
A facile method was developed for the synthesis of quinazolinone derivatives in a one-pot condensation reaction via in situ amine generation using ammonia as the amine source and with the formation of four new C–N bonds in good to excellent yields. With the optimised method, we synthesized a library of piperazine linked quinazoline derivatives and the synthesized compounds were evaluated for their inhibitory activity against Mycobacterium tuberculosis. The compounds 8b, 8e, 8f, 8m, 8n and 8v showed potent anti-mycobacterial activity with MIC values of 2–16 μg mL−1. All the synthesized compounds follow Lipinski''s rules for drug likeness.A facile method was developed for the synthesis of quinazolinone derivatives in a one-pot condensation reaction via in situ amine generation using ammonia as the amine source and with the formation of four new C–N bonds in good to excellent yields. 相似文献
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Carbon dioxide (CO2) is a greenhouse gas whose presence in the atmosphere significantly contributes to climate change. Developing sustainable, cost-effective pathways to convert CO2 into higher value chemicals is essential to curb its atmospheric presence. Electrochemical CO2 reduction to value-added chemicals using molecular catalysis currently attracts a lot of attention, since it provides an efficient and promising way to increase CO2 utilization. Introducing amino groups as substituents to molecular catalysts is a promising approach towards improving capture and reduction of CO2. This review explores recently developed state-of-the-art molecular catalysts with a focus on heterogeneous and homogeneous amine molecular catalysts for electroreduction of CO2. The relationship between the structural properties of the molecular catalysts and CO2 electroreduction will be highlighted in this review. We will also discuss recent advances in the heterogeneous field by examining different immobilization techniques and their relation with molecular structure and conductive effects.Electroreduction of CO2 to CO using molecular catalysis. 相似文献
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Lindsay N. Avolio Tyler J. S. Smith Ana Navas-Acien Kate Kruczynski Nora Pisanic Pranay R. Randad Barbara Detrick Rebecca C. Fry Alexander van Geen Walter Goessler Ruth A. Karron Sabra L. Klein Elizabeth L. Ogburn Marsha Wills-Karp Kelsey Alland Kaniz Ayesha Brian Dyer Md. Tanvir Islam Habibat A. Oguntade Md. Hafizur Rahman Hasmot Ali Rezwanul Haque Saijuddin Shaikh Kerry J. Schulze A. K. M. Muraduzzaman A. S. M. Alamgir Meerjady S. Flora Keith P. West Jr. Alain B. Labrique Christopher D. Heaney for the JiVitA Maternal Child Health Nutrition Research Project 《Paediatric and perinatal epidemiology》2023,37(2):165-178
Background
Arsenic exposure and micronutrient deficiencies may alter immune reactivity to influenza vaccination in pregnant women, transplacental transfer of maternal antibodies to the foetus, and maternal and infant acute morbidity.Objectives
The Pregnancy, Arsenic, and Immune Response (PAIR) Study was designed to assess whether arsenic exposure and micronutrient deficiencies alter maternal and newborn immunity and acute morbidity following maternal seasonal influenza vaccination during pregnancy.Population
The PAIR Study recruited pregnant women across a large rural study area in Gaibandha District, northern Bangladesh, 2018–2019.Design
Prospective, longitudinal pregnancy and birth cohort.Methods
We conducted home visits to enrol pregnant women in the late first or early second trimester (11–17 weeks of gestational age). Women received a quadrivalent seasonal inactivated influenza vaccine at enrolment. Follow-up included up to 13 visits between enrolment and 3 months postpartum. Arsenic was measured in drinking water and maternal urine. Micronutrient deficiencies were assessed using plasma biomarkers. Vaccine-specific antibody titres were measured in maternal and infant serum. Weekly telephone surveillance ascertained acute morbidity symptoms in women and infants.Preliminary Results
We enrolled 784 pregnant women between October 2018 and March 2019. Of 784 women who enrolled, 736 (93.9%) delivered live births and 551 (70.3%) completed follow-up visits to 3 months postpartum. Arsenic was detected (≥0.02 μg/L) in 99.7% of water specimens collected from participants at enrolment. The medians (interquartile ranges) of water and urinary arsenic at enrolment were 5.1 (0.5, 25.1) μg/L and 33.1 (19.6, 56.5) μg/L, respectively. Water and urinary arsenic were strongly correlated (Spearman's ⍴ = 0.72) among women with water arsenic ≥ median but weakly correlated (⍴ = 0.17) among women with water arsenic < median.Conclusions
The PAIR Study is well positioned to examine the effects of low-moderate arsenic exposure and micronutrient deficiencies on immune outcomes in women and infants. Registration : NCT03930017. 相似文献79.
Misha?TeimouriEmail author Md?Salleh?Hassan Mark?Griffiths Seyed?Rahim?Benrazavi Jusang?Bolong Azlina?Daud Nor?Azura?Adzharuddin 《Child indicators research》2016,9(2):407-428
Before the advent of the Internet, television and film was the only audio-visual medium to which most children were exposed. The risks were primarily limited to children being exposed to sexual and violent materials, the nature of which were known and easy to control. Nowadays, children are surrounded by a variety of digital media and are exposed to different risks, many of which are still unknown. The Internet is fully integrated into children’s daily lives, along with the potential risks. The present study aimed to (i) describe the level of risks children are exposed to, and (ii) test the measurement validity of a total of 45 items assessing nine scales of online risk to children that were adapted from studies carried out in Europe and the United States. The study comprised 420 schoolchildren. The results showed that children were more exposed to ‘unwanted exposure to pornography’ and less to ‘conduct risk’ (e.g., accidental illegal downloading; creating profiles on inappropriate websites). Boys and older children were more exposed to the risks compared to girls and younger children. The study validated five dimensions (inappropriate materials, sexting, contact-related risks, risky online sexual behavior, and bullying/being bullied) assessing online risk to children by using exploratory and confirmatory factor analyses. The study found that scales developed in Europe and the United States are not wholly suitable to an Asian context and needed to be modified. Further investigation to classify online risks to children and offer a solutions to reducing the online risks. 相似文献
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