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Gaucher disease in Montenegro-genotype/phenotype correlations:Five cases report

World Journal of Clinical Cases 2019年第12期7卷 1475-1482页

作者： Snezana Vujosevic Sanja Medenica Vesko Vujicic Milena Dapcevic nikola bakic Ruhua Yang Jun Liu Pramod K Mistry Department of Endocrinology Internal Medicine Clinic Clinical Center of Montenegro Faculty of Medicine University of Montenegro Podgorica 81000 Montenegro Hematology Department Internal Medicine Clinic Clinical Center of Montenegro Faculty of Medicine University of Montenegro Podgorica 81000 Montenegro Yale Lysosomal Disease Center and Inherited Metabolic Liver Disease Clinic Yale University School of Medicine New Haven CT 06510 United States

BACKGROUND The most common lysosomal storage disorder is Gaucher disease (GD). It is a deficiency of lysosomal glucocerebrosidase (GBA) due to biallelic mutations in the GBA gene, characterized by the deposition of glucocerebroside in macrophage-monocyte system cells. The report targets clinical phenotypes of GD in order to correlate them with GBA gene mutations, as well as to identify GBA gene mutation in patients in Montenegro that are diagnosed with GD. CASES SUMMARY Five patients (4 male, 1 female) of type 1 GD (GD1) are reported. The age at diagnosis ranged from 7 to 40. Patients experienced delays of 1-12 years in diagnosis after the original onset of symptoms. The most common mode of presentation was a variable degree of splenomegaly and thrombocytopenia, while other symptoms included bone pain, hepatomegaly, abdominal pain and fatigue. Osteopenia was present in a majority of the patients: 4/5. All patients were found to have an asymptomatic Erlenmeyer flask deformity of the distal femur. On enzyme replacement therapy (ERT), the hematological and visceral parameters showed significant improvement, but no significant progression in bone mineral density was noticed. GBA gene sequencing revealed homozygosity for the N370S mutation in one patient. The genotypes of the other patients were N370S/55bp deletion, N370S/D409H (2 patients), and H255Q/N370S (1 patient). CONCLUSION The phenotypes of the GD1 encountered in Montenegro were severe but all responded well to ERT.

关键词： Gaucher disease Lysosomal storage disorder Glucocerebrosidase GBA gene sequencing Genotype Case report

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Role of copper chelating agents: between old applications and new perspectives in neuroscience

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Neural Regeneration Research 2025年第3期20卷 751-762页

作者： Rosalba Leuci Leonardo Brunetti Vincenzo Tufarelli Marco Cerini Marco Paparella nikola Puvača Luca Piemontese Department of Pharmacy-Pharmaceutical Science University of Bari Aldo MoroBariItaly Department of Precision and Regenerative Medicine and Jonian Area(DiMePRe-J) Section of Veterinary Science and Animal ProductionUniversity of Bari Aldo MoroBariItaly Department of Engineering Management in Biotechnology Faculty of Economics and Engineering Management in Novi SadUniversity Business Academy in Novi SadNovi SadSerbia

The role of copper element has been an increasingly relevant topic in recent years in the fields of human and animal health, for both the study of new drugs and innovative food and feed supplements. This metal plays an important role in the central nervous system, where it is associated with glutamatergic signaling, and it is widely involved in inflammatory processes. Thus, diseases involving copper(Ⅱ) dyshomeostasis often have neurological symptoms, as exemplified by Alzheimer's and other diseases(such as Parkinson's and Wilson's diseases). Moreover, imbalanced copper ion concentrations have also been associated with diabetes and certain types of cancer, including glioma. In this paper, we propose a comprehensive overview of recent results that show the importance of these metal ions in several pathologies, mainly Alzheimer's disease, through the lens of the development and use of copper chelators as research compounds and potential therapeutics if included in multi-target hybrid drugs. Seeing how copper homeostasis is important for the well-being of animals as well as humans, we shortly describe the state of the art regarding the effects of copper and its chelators in agriculture, livestock rearing, and aquaculture, as ingredients for the formulation of feed supplements as well as to prevent the effects of pollution on animal productions.

关键词： agriculture Alzheimer's disease chelators copper feed supplements multi-target

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Classical behaviour of various variables in an open Bose-Hubbard system

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Chinese Physics B 2011年第12期20卷 63-71页

作者： nikola Buri Institute of Physics University of BelgradeP O BOX 6811000 BelgradeSerbia

Quantum dispersions of various sets of dynamical variables of an open Bose-Hubbard system in a classical limit are studied. To this end, an open system is described in terms of stochastic evolution of its quantum pure states. It is shown that the class of variables that display classical behaviour crucially depends on the type of noise. This is relevant in the mean-field approximation of open Bose-Hubbard dynamics.

关键词： quantum trajectories decoherence classical limit

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Glial cells in intracerebral transplantation for Parkinson’s disease

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Neural Regeneration Research 2020年第7期15卷 1173-1178页

作者： nikola Tomov Institute of Anatomy University of Bern

In the last few decades,intracerebral transplantation has grown from a dubious neuroscientific topic to a plausible modality for treatment of neurological *** possibility for cell replacement opens a new field of perspectives in the therapy of neurodegenerative disorders,ischemia,and neurotrauma,with the most lessons learned from intracerebral transplantation in Parkinson's *** animal studies and a few small-scale clinical trials have proven the concept of intracerebral grafting,but still have to provide a uniform and highly efficient approach to the procedure,suitable for clinical *** success of intracerebral transplantation is highly dependent on the integration of the grafted cells with the host *** this process,glial cells are clearly more than passive *** provide transplanted cells with mechanical support,trophics,mediate synapse formation,and participate in graft *** the same time,glial cells mediate scarring,graft rejection,and neuroinflammation,which can be *** can use this information to try to understand the mechanisms behind the glial reaction to intracerebral *** and utilizing glial reactivity can move translational research forward and provide an insight not only to post-transplantation events but also to mechanisms of neuronal death and *** about glial reactivity to transplanted cells could also be a key for optimization of transplantation protocols,which ultimately should contribute to greater patient benefit.

关键词： astroglia dopaminergic glial scarring microglia neuroinflammation Parkinson's disease transplantation

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A comparison of power measurement techniques and electrical characterization of an atmospheric pressure plasma jet

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Plasma Science and Technology 2022年第10期24卷 74-85页

作者： Olivera JOVANOVIĆ Nevena PUAČ nikolaŠKORO Institute of Physics University of BelgradePregrevica 118Belgrade 11080Serbia

In the last two decades a growing interest has been shown in the investigation of atmospheric pressure plasma jets(APPJs)that operate in contact with liquid *** order to form a complete picture about such experimental systems,it is necessary to perform detailed diagnostics of plasma jets,as one step that will enable the adjustment of system properties for applications in different *** this work,we conducted a detailed electrical characterisation of a plasma system configuration used for water treatment.A helium plasma jet,with a pin electrode powered by a continuous sine wave at a frequency of 330 k Hz,formed a streamer that was in contact with a distilled water *** electrical circuit allowed the monitoring of electrical signals supplied to the jet and also to the plasma *** electrical characterisation together with power consumption measurements was obtained by using two different *** first method was based on the direct measurements of voltage and current signals,while in the second method we used'Lissajous figures'.We compared these two methods when used for discharge power estimation and addressed their advantages and *** results showed that both of these methods could be used to successfully determine power consumed by a discharge in contact with water,but only when taking into account power dissipation without plasma.

关键词： APPJ electrical characterization discharge power Lissajous figure

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Using antifibrinolytics to tackle neuroinflammation

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Neural Regeneration Research 2020年第12期15卷 2203-2206页

作者： Stanimir Atsev nikola Tomov Faculty of Medicine Trakia UniversityStara ZagoraBulgaria Institute of Anatomy University of BernBernSwitzerland

Plasmin is generally known as a promotor of *** advancement suggests that it has a complex role as immunity *** inhibition of plasmin production and activity has been proven to improve neurological outcomes in traumatic brain injury and subarachnoid hemorrhage,most probably by preventing *** immune-modulatory properties of antifibrinolytics,however,suggest that they probably have effects unrelated to fibrinolysis inhibition,which are currently not adequately *** present work aims to give an account of the existing data regarding antifibrinolytics as agents influencing *** and clinical studies on the possible influence of antifibrinolytics on neuroinflammation are ***,the emerging evidence suggests that inhibition of plasmin(ogen)activity can ameliorate neuroinflammation to some *** data demonstrate that plasmin(ogen)is not exclusively involved in fibrinolysis,but also has other substrates and can precipitate in inflammatory *** on the role of plasmin as the factor for the development of neuroinflammation shows the significant potential of antifibrinolytics as pharmacotherapy of neuroinflammationm,which is worthy of further exploration.

关键词： antifibrionolytics fibrin fibrinogen neuroinflammation plasmin plasminogen tranexamic acid

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ON CRITERIA FOR STARLIKENESS AND CONVEXITY OF ANALYTIC FUNCTIONS

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Acta Mathematica Scientia 2004年第4期24卷 597-602页

作者： Vikramaditya Singh nikola Tuneski Faculty of Mechanical Engineering Karpos Ⅱ b.b. 1000 Skopje Republic of MacedoniaFaculty of Mechanical Engineering Karpos Ⅱ b.b. 1000 Skopje Republic of Macedonia

In this paper the authors study (1-γ+zf(?)(z)/f'(z))/(zf'(z)/f(z)) as a criteria for starlikeness and convexity. Sharp upper bound of |α2| and of the Fekete-Szego functional |α3-μα22| is given for a class of anal... 详细信息

关键词： Starlike convex criteria Fekete-Szego

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Alternative routes to equivalent classical models of a quantum system

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Chinese Physics B 2012年第12期21卷 51-56页

作者： M.Radonji Slobodan Prvanovi nikola Buri Institute of Physics University of Belgrade

Coarse-graining of some sort is a fundamental and unavoidable step in any attempt to derive the classical mechanical behavior from the quantum *** utilize the two-mode Bose-Hubbard model to illustrate how different coarse-grained systems can be naturally associated with a fixed quantum system if it is compatible with different dynamical *** coarse-grained systems generate different evolutions of the same physical quantities,and the difference becomes negligible only in the appropriate macro-limit.

关键词： two-mode Bose-Hubbard model coarse-grained system Hamilton operator system dynamical algebra

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Dynamical time versus system time in quantum mechanics

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Chinese Physics B 2012年第7期21卷 101-107页

作者： Duan Arsenovi nikola Buri Dragomir Davidovi Slobodan Prvanovi Institute of Physics University of Belgrade Vinca Institute of Nuclear Sciences

Properties of an operator representing the dynamical time in the extended parameterization invariant formulation of quantum mechanics are studied. It is shown that this time operator is given by a positive operator measure analogously to the quantities that are known to represent various measurable time operators. The relation between the dynamical time of the extended formulation and the best known example of the system time operator, i.e., for the free one- dimensional particle, is obtained.

关键词： time operators extended state space positive operator valued measure

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Synchronization of the minimal models of bursting neurons coupled by delayed chemical or electrical synapses

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Chinese Physics B 2012年第1期21卷 29-36页

作者： Neboja Vasovi nikola Buri Kristina Todorovi Ines Grozdanovi Department of Applied Mathematics Faculty of Mining and GeologyBelgrade 162 Serbia Institute of Physics University of Beograd PO Box 68 11080 Beograd-Zemun Serbia Department of Physics and Mathematics Faculty of Pharmacy University of Belgrade Vojvode Stepe 450 Belgrade Serbia

The minimal two-dimensional model of bursting neuronal dynamics is used to study the influence of time-delay on the properties of synchronization of bursting neurons. Generic properties of bursting and dependence of the stability of synchronization on the time-lag and the strength of coupling are described, and compared with the two common types of synaptical coupling, i.e., time-delayed chemical and electrical synapses.

关键词： neuronal nursting minimal model synchronization

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