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Juvenile hemochromatosis:HAMP mutation and severe iron overload treated with phlebotomies and deferasirox

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World Journal of clinical cases 2017年第10期5卷 381-383页

作者： Manuel A Lescano Letícia c Tavares Paulo c J L Santos Institute of digestive Tract of Southwestern Bahia Laboratory of Genetics and Molecular cardiology Heart Institute (InCor)University of Sao Paulo Medical School

Juvenile hemochromatosis(JH) is a rare condition classified as an autosomal recessive disorder that leads to severe iron absorption. JH usually affects people under the age of 30 and presents symptoms such as chronic liver damage,hypogonadotropic hypogonadism,cardiac diseases and endocrine dysfunctions. The present case reports a 29-year-old Brazilian woman with JH condition due to HAMP mutation(g.47G>A),treated with phlebotomies and deferasirox. She presented symptoms such as weakness,skin hyperpigmentation,joint pain in the shoulders and hands and amenorrhea. First laboratory tests showed altered biochemical parameters [serum ferritin(SF): 5696 ng/mL,transferrin saturation(TS): 85%]. After sessions of phlebotomies(450 mL every 15 d),the patient presented partial symptomatic improvements and biochemical parameters(SF: 1000 ng/mL,Hb: 11 g/dL). One year later,deferasirox(15 mg/kg per day) was introduced to the treatment,and the patient showed total symptomatic improvement,with significant clearing of the skin,SF: 169 ng/mL,and TS: 50%. Furthermore,after the combined deferasirox-phlebotomy therapy,magnetic resonance imaging measurements revealed normalized level for liver iron(30 μmol/g; reference value conclusion,combined deferasirox-phlebotomy treatment was able to normalize iron levels and improve symptoms.

关键词： Genetic disease Juvenile hemochromatosis HAMP gene Mutation Iron chelation

Evidence for a female-produced, long range pheromone of Anoplophora glabripennis （coleoptera cerambycidae）

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Insect Science 2012年第3期19卷 355-371页

作者： Jacob d.Wickham Zhichun Xu Stephen A.Teale department of Environmental and Forest Biology SUNY College of Environmental Science and Forestry1 Forestry DriveSyracuseN Y 13210USA college of Forest Resources Beijing Forestry University Key Lab of Analytical chemistry for Living Biosystems Institute of ChemistryChinese Academy of SciencesBeijingChina

Volatiles from female Asian longhorned beetle （ ALB ）, Anoplophora glabripenhis, were evaluated as candidate sex pheromone components. Previous studies on ALB have revealed several antennally active compounds from virgin females; however the origins and activity of these compounds were not apparent and require further investigation. We tested the hypothesis that one or more of the ALB contact sex pheromones is a precursor that undergoes abiotic oxidation to yield volatile pheromone components, and evaluated the activity of these compounds using laboratory and field bioassays. Gas chromatogra- phy coupled electroantennography detection （Gc-EAd） analysis indicated the presence of three antennally active aldehydes （heptanal, nonanal, and hexadecanal） in female cuticular extracts exposed to ozone or UV and visible light. In laboratory bioassays using a Y-tube olfactometer, males were preferentially attracted to ozonized female body washes over crude body washes. Similarly, synthetic formulations of these compounds were preferred over controls in the olfactometer. Field trapping experiments conducted from 2006 to 2008 in Ningxia, china showed that synthetic lures of the three aldehydes formulated in a ratio simulating that of virgin females attracted more beetles compared to controls, and that combinations of these aldehydes, linalool oxide, and host kairomones captured more beetles than controls, and captured significantly more males.

关键词： Anoplophora glabripennis Asian longhorned beetle cuticular hydrocarbons,kairomone oxidation pheromone

Time-dependent Early-age Behaviors of concrete under Restrained condition

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Journal of Wuhan University of Technology(Materials Science) 2007年第2期22卷 350-353页

作者：马新伟 R d Hooton H Lam School of Materials Science and Engineering Harbin Institute of Technology department of civil Engineering University of Toronto

To investigate the early-age behaviors of concrete under a restrained condition, a set of apparatus was developed. In this way, the tensile creep and other early-age properties can be investigated in depth. By measuring the modulus of elasticity of concrete, synchronous shrinkage of concrete and steel rings and free shrinkage of concrete, the deformations of concrete ring can be quantified respectively. The experimental results show the tensile stress in concrete is time-dependent, and the stress at cracking is much lower than the tensile strength at that age; the tensile creep plays an important role in relaxing the tensile stress and postponing the cracking of concrete.

关键词： shrinkage restrained shrinkage elastic strain tensile creep time-dependent behaviors

Small-World Networks with Unitary cayley Graphs for Various Energy Generation

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computer Systems Science & Engineering 2023年第6期45卷 2773-2782页

作者： c.Thilag P.B.Sarasija department of Mathematics Noorul Islam Centre for Higher EducationKumaracoil629180India

complex networks have been a prominent topic of research for several years,spanning a wide range of fields from mathematics to computer science and also to social and biological *** eigenvalues of the Seidel matrix,Seidel Signless Laplacian matrix,Seidel energy,Seidel Signless Laplacian energy,Maximum and Minimum energy,degree Sum energy and distance degree energy of the Unitary cayley graphs[UcG]have been ***-power devices must be able to transfer data across long distances with low delay and *** overcome this drawback a small-world network depending on the unitary cayley graph is proposed to decrease the delay and increase the reliability and is also used to create and analyze network ***-world networks based on the cayley graph have a basic construction and are highly *** simulation result shows that the small-world network based on unitary cayley graphs has a shorter delay and is more ***,the maximum delay is lowered by 40%.

关键词： Seidel energy Seidel Signless Laplacian eigenvalues distance degree energy Unitary cayley graphs

Trace elements migrating from tailings to rock varnish laminated sediments in an old mining region from Nelson,Nevada,USA

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International Journal of Sediment Research 2022年第2期37卷 202-213页

作者： douglas B.Sims Amanda c.Hudson John E.Keller Michael Strange Andressa cristhy Buch david Ferrari Giavanna M.Fernandez Juan Garcia-Hernandez Bailey d.Kesl Sean Torres department of Physical Sciences College of Southern Nevada University of Nevada Fluminense Federal University

The dispersion and influence of soluble and particulate trace elements(TEs) present in the materials from abandoned mines is an imminent problem on terrestrial and aquatic *** goal of this research is to provide a broader view of such transport,assessing particle size distribution and TE interactions(e.g.,scavenging) with mine tailings and locally derived sediments transported by water in arid *** grains from wash sediment were collected from a dry ephemeral wash in Nelson,Nevada where the Techatticup Mine and Mill worked between 1850 and *** were evaluated to determine the association and behavior of certain metals and metalloids including aluminum,arsenic,barium,cadmium,copper,chromium,iron,manganese,lead,selenium,vanadium,and zinc to rock varnish laminated *** show that some metals and metalloids were more concentrated on larger particles whereas others indicated the opposite *** example,As was greatest on silt fractions and least on coarse fractions,while Se was detected only on ***,cu,Fe,Mn,Pb,and Zn concentrations all increased with decreasing particle size(silt> sand> coarse),whereas Al,Ba,and V showed the opposite trend(silt d coarse).The enrichment factor(EF) of cu,Zn,Pb,As,and Se were lower in mine tailing sediment treated with wash than those obtained for source *** in treated wash sediment samples,the high EFs of Ba,cr,V,and Mn suggested a possible greater affinity of these metals to the Al,Fe,and Mn oxides from the RV *** data found in this study show that transport of contaminated sediments in arid regions could lead to the enrichment of some TEs in downgradient areas from source materials.

关键词： Adsorption Metals Metalloids Mine tailings Particle size Transport

SERIneALanine Killer:SPT promiscuity inhibits tumour growth via intra-tumoral deoxysphingolipid production

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Signal Transduction and Targeted Therapy 2020年第1期5卷 219-220页

作者： Mattia Falcone Oliver d.K.Maddocks University of Glasgow Institute of cancer Sciences Wolfson Wohl Cancer Research CentreGarscube EstateGlasgow G611QHUK

In a recent article in Nature,Muthusami et al.1 highlight the promiscuity of serine palmitoyltransferase(SPT)for non-essential amino acids under low serine conditions,illustrating a previously unappreciated mechanism ... 详细信息

关键词： Killer SPT tumour

Marine Metagenome as A Resource for Novel Enzymes

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Genomics, Proteomics & Bioinformatics 2015年第5期13卷 290-295页

作者： Amani d.Alma'abadi Takashi Gojobori Katsuhiko Mineta computational Bioscience Research center (cBRc) King Abdullah University of Science and Technology (KAUST)

More than 99% of identified prokaryotes, including many from the marine environment, cannot be cultured in the laboratory. This lack of capability restricts our knowledge of microbial genetics and community ecology. Metagenomics, the culture-independent cloning of environmental dNAs that are isolated directly from an environmental sample, has already provided a wealth of information about the uncultured microbial world. It has also facilitated the discovery of novel bio- catalysts by allowing researchers to probe directly into a huge diversity of enzymes within natural microbial communities. Recent advances in these studies have led to a great interest in recruiting microbial enzymes for the development of environmentally-friendly industry. Although the metage- nomics approach has many limitations, it is expected to provide not only scientific insights but also economic benefits, especially in industry. This review highlights the importance of metagenomics in mining microbial lipases, as an example, by using high-throughput techniques. In addition, we dis- cuss challenges in the metagenomics as an important part of bioinformatics analysis in big data.

关键词： Microbial diversity culture-independent studies catalysis Lipase Biotechnology

Joins of 1-Planar Graphs

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Acta Mathematica Sinica,English Series 2014年第11期30卷 1867-1876页

作者： Július cZAP dvid HUdK Tom MAdARAS department of Applied Mathematics and Business Informatics Faculty of Economics Technical University of KoiceNěmcovej 32040 01 KoiceSlovakia VSL Software a.s.Lomen 8040 01 KoiceSlovakia Institute of Mathematics Faculty of SciencePavol Jozef afrik University Jesenn 5040 01 KoiceSlovakia

A graph is called 1-planar if it admits a drawing in the plane such that each edge is crossed at most *** this paper,we study 1-planar graph *** prove that the join G ＋ H is 1-planar if and only if the pair [G,H] is subgraph-majorized by one of pairs [c3 ∪ c3,c3],[c4,c4],[c4,c3],[K2,1,1,P3] in the case when both elements of the graph join have at least three *** one element has at most two vertices,then we give several necessary/sufficient conditions for the bigger element.

关键词： 1-Planar graph join product

TSKARNA-norm Adaption Based NLMS with Optimized Fractional Order PId controller Gains for Voltage Power Quality

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chinese Journal of Electrical Engineering 2023年第3期9卷 84-98页

作者： Prashant Kumar Sabha Raj Arya Khyati d.Mistry department of Electrical Engineering Sardar Vallabhbhai National Institute of TechnologySurat 395007India

The operation of a dynamic voltage restorer(dVR)is studied using a three-phase voltage source converter(VSc)-based topology to alleviate voltage anomalies from a polluted supply *** control algorithm used included two *** first is an adaptive Takagi-Sugeno-Kang(TSK)-based adaptive reweighted L1 norm adaption-based normalized least mean square(TSK-ARNA-NLMS)unit,which is proposed for the extraction of fundamental active and reactive components from the non-ideal supply and is further employed to generate the load reference voltage and switching pulse for the *** step size was evaluated using the proposed TSK-ARNA-NLMS controller,and the TSK unit was optimized by integration with the marine predator algorithm(MPA)for a faster convergence *** second,a fractional-order PId controller(FOPId),was employed for Ac-and dc-link voltage regulation and was approximated using the Oustaloup *** FOPId()PI dγμprovides more freedom for tuning the settling time,rise time,and *** FOPId coefficients(Ki,Kd,Kp,γ,andμ)were optimized by employing an advanced ant lion optimization(ALO)meta-heuristics technique to minimize the performance index,namely,the integral time absolute error(ITAE)and assess the accuracy of *** dVR performance was validated under dynamic-and steady-state conditions.

关键词： Takagi-Sugeno-Kang(TSK) ARNA-NLMS FOPId-ALO unbalances gain optimization approximation Oustaloup technique