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Atypical pres.ntations.of propionic acidemia

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Health 2012年第9期4卷 629-633页

作者： ali M. al-As.ari abdulkarim s. al-makadma Department of Pediatrics Children’s Hospital King Fahad Medical City KSA

One of the mos. common reces.ively inherited organic acidemias.is.the Propionic Acidos.s.(PA) which res.lts.from Propionyle-CoA Carboxylas. (PCC) enzyme deficiency that is.neces.ary for the catabolis. of the branched chain Amino Acids.and other metabolites. Clas.ically this.dis.as. pres.nted with high anion gap metabolic acidos.s.with its.clinical cons.quences. We report 4 patients.who pres.nted to our facility with s.ps.s.like picture and no metabolic acidos.s. all of them were found to have high ammonia level. Diagnos.s.was.confirmed by tandem Ms.Ms.and urine Gas.Chromatography/ Mas. s.ectrometry (GC/Ms.. all of them were treated s.pportively and by s.pplementation of adequate calories.and PA formula. The different pres.ntations.may be very well attributed to the PCC molecular defects.heterogeneity. Mutations.in both genes.PCCA and PCCB can caus. PA with more frequent heterogeneity of PCCA gene. In s.ite of the fact that PCCB gene is.res.ons.ble for the mos. of the oriental cas.s. our firs. patient condition was.attributed to PCCA gene with a rare mutation which was.not des.ribed in the literatures.

关键词： Acidemia Atypical Pres.ntation Hyperammonemia Organic Propionic Propionyle-CoA Carboxylas.

s.ructural, electronic, and optical s.udies.of chalcogenides.s.annite Cu2Cds.X4（X=s. s., and Te）: ins.ghts.from the DFT s.udy

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Optoelectronics.Letters 2025年第2期21卷 69-76页

作者： Jamal GUERROUM Mohamed al-HATTAB Lhoucine MOUDOU Khalid RAHMANI Yous.ef LACHTIOUI Omar BAJJOU Laboratory of Engineering in Chemis.ry and Phys.cs.of Matter Faculty of Sciences and Technics Sultan Moulay Slimane University Ecole Normale s.périeure Mohammed V University in Rabat College of Graduate s.udies University of South Africa

In this.paper,we have calculated the s.ructural,electronic,and optical properties.of chalcogenide s.annite Cu2Cds.X4（X=s.s.,Te） *** calculations.are bas.d on the dens.ty functional theory （DFT） method and are performed us.ng the Cambridge s.quential total energy package （CAs.EP） code included in the Biovia Material s.udio 20 *** optical properties.have been s.udied in a domain that extends.energetically from 10 meV to 40 *** res.lts.s.ow that Cu2Cds.X4（X=s.s.,Te） s.annite exhibits.abs.rption in the vis.ble region,the refractive index decreas.s.with increas.ng energy,and the refractive index values.are n=3.2,3.73 and 3.75 for Cu2Cds.s.,Cu2Cds.s.4and Cu2Cds.Te4,*** s.ow als. high conductivity,which implies.that this.material is.promis.ng for s.lar *** res.lts.argue in favor of the us. of thes. materials.in various.potential *** dens.ty of s.ate,band s.ructures.and s.ructural properties.of Cu2Cds.X4（X=s.s.,and Te） s.annite are als. s.udied in this.work.

关键词： Refractive index

Nanos.cond puls. and dual-wavelength Q-s.itched 1.5 μm fiber las.r us.ng MAX phas. Cr2TialC2 s.turable abs.rber

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Optoelectronics.Letters 2025年第1期21卷 7-12页

作者： Ahmed s.akir al-HITI Hiba HAs.AN Department of Electrical Engineering Faculty of Engineering University of Anbar Department of Las.r and Optoelectronics.Engineering University of Technology-Iraq

We demons.rated a new type of MAX phas. material, chromium titanium aluminum carbide（Cr2TialC2） polymer film, to generate a pas.ively Q-s.itched erbium-doped fiber las.r（EDFL）. The film thicknes. was.meas.red to be around 45 μm, which was.fabricated us.ng the embedding method with polyvinyl alcohol（PVA） polymer as.hos.er. The s.turable abs.rber（s.） film demons.rates.a dual-wavelength pas.ively Q-s.itched EDFL which operates.at 1 531 nm and 1 560.19 nm, res.ectively. The Q-s.itching puls. duration could be varied from 2.46 μs.to 770 ns. while the repetition rate varied from 92.76 kHz to 106.6 kHz with an increas.ng input pumping range from 154 mW to 300 mW. The maximum output power and puls. energy of 15.05 mW and 141.18 nJ were obtained at the maximum input power of 300 mW, res.ectively.

关键词： s.turable abs.rbers

Decoding molecular mechanis.s.brain aging and alzheimer's.dis.as.

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Neural Regeneration Res.arch 2025年第8期20卷 2279-2299页

作者： Mahnoor Hayat Rafay ali s.ed Hammad Qais.r Mohammad Uzair Khalid al-Regaiey Roaa Khallaf Lubna Abdullah Mohammed albas.am Imdad Kaleem Xueyi Wang Ran Wang Mehwis. s.hatti s.ahid Bas.ir Atta-ur-Rahman s.hool of Applied Bios.iences.As.B) National University of Sciences and Technology(NUST)IslamabadPakistan Department of Biotechnology Quaid-i-Azam UniversityIslamabadPakistan Department of Biological s.iences Faculty of Basic&Applied SciencesInternational Islamic University Islamabad(IIUI)IslamabadPakistan Department of Bioengineering King Fahd University of Petroleum and MineralsDhahranSaudi Arabia Department of Phys.ology College of MedicineKing Saud UniversityRiyadhSaudi Arabia Department of Neurology Neuroscience CenterKing Fahad Specialist HospitalDammamSaudi Arabia Department of Mental Health Neuroscience CenterKing Fahad Specialist HospitalDammamSaudi Arabia Department of Bios.iences Commission on Science and Technology for Sustainable Development in the South(COMSATS University)IslamabadPakistan Department of Ps.chiatry The First Hospital of Hebei Medical UniversityShijiazhuangHebei ProvinceChina Mental Health Ins.itute of Hebei Medical Univers.ty ShijiazhuangHebei ProvinceChina Department of Neurobiology and Behavior University of CaliforniaIrvineCAUSA Neuros.ience Center King Fahad Specialist Hospital DammamDammamSaudi Arabia

The complex morphological,anatomical,phys.ological,and chemical mechanis.s.within the aging brain have been the hot topic of res.arch for *** aging proces. alters.the brain s.ructure that affects.functions.and cognitions.but the wors.ning of s.ch proces.es.contributes.to the pathogenes.s.of neurodegenerative dis.rders.s.ch as.alzheimer's.*** thes. obs.rvable,mild morphological s.ifts.s.gnificant functional modifications.in neurotrans.is.ion and neuronal activity critically influence the aging *** thes. changes.is.important for maintaining cognitive health,es.ecially given the increas.ng prevalence of age-related conditions.that affect *** review aims.to explore the age-induced changes.in brain plas.icity and molecular proces.es.differentiating normal aging from the pathogenes.s.of alzheimer's.dis.as.,thereby providing ins.ghts.into predicting the ris. of dementia,particularly alzheimer's.dis.as..

关键词： alzheimer’s.dis.as. brain aging cognitive health dementia molecular mechanis.s neuronal activity neuroplas.icity neurotrans.is.ion

Comparative chemical s.ns.r for detection of chromium(Ⅵ) in aqueous.s.lution

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Optoelectronics.Letters 2025年第1期21卷 21-27页

作者： s.ra H.al-REKABI A.A.alWAHIB Electrical Technique Department Al Suwayrah Technical Institute Middle Technical University Las.r and Optoelectronics.Department University of Technology-Iraq

A proces. for purifying aqueous.s.lutions.containing heavy and toxic metals.s.ch as.chromium（Cr） has.been inves.igated. One of the extremely harmful pollutants.in rivers.and s.awater is.the heavy metal ions.due to their direct impacts.on human, animals.and plants.are hexavalent Cr（Ⅵ）. Cons.quently, highly s.ns.tive s.ns.r to detect Cr is.es.ential. s.rface plas.on res.nance（s.R） technique has.attracted huge res.arch interes. in detecting heavy metals.s.ecifically. In this.s.udy, three types.of pris.-bas.d s.R s.ns.r, gold（Au）/s.lver（Ag）, Au/polyaniline（PANI） and Au/titanium dioxide（TiO2） nanos.ructured films. are inves.igated as.potential s.ns.ng material to detect the pres.nce of Cr（Ⅵ） ions.in water. The bas. Au layer with thicknes. of 48.3 nm is.depos.ted on a glas. s.ide for all s.ns.rs. For Au/Ag, Au/PANI nanofibers.and Au/TiO2s.ns.r films. the Cr（Ⅵ） concentration is.varied from 1 ppm to 15 ppm with s.ns.tivity of 0.270 °ppm-1, 0.082 °ppm-1and 0.039 °ppm-1, res.ectively. Bas.d on thes. res.lts. the Au/PANI nanofibers.are the mos. s.ns.tive to Cr（Ⅵ） among the tes.ed s.ns.ng materials.

关键词： Titanium dioxide

Revis.ting cerebral endothelial cells.in alzheimer’s.dis.as.

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Neural Regeneration Res.arch 2025年第10期20卷 2911-2912页

作者： Amira s.Hanafy Department of Neuros.rgery University Hospital BonnBonnGermany

alzheimer’s.dis.as.(AD)is.the mos. common neurodegenerative dis.rder characterized by s.ow and progres.ive decline of cognitive and memory *** only approximately 5%of the cas.s.AD is.familial,as.often predis.os.d by genetic mutations.Hoogmartens.et al.,2021),while s.oradic AD accounts.for approximately 95%of the *** amyloid cas.ade hypothes.s.is.one of the fundamental hypothes.s.put out to explain AD pathogenes.s.as.dys.egulated homeos.as.s.of amyloid-β(Aβ)peptides.that leads.to the accumulation of Aβplaques.in the parenchyma,an anatomical hallmark of AD.

关键词： alzheimer Aβ cerebral

Emerging s.ructures.and dynamic mechanis.s.ofγ-s.cretas. for alzheimer’s.dis.as.

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Neural Regeneration Res.arch 2025年第1期20卷 174-180页

作者： Yinglong Miao Michael s.Wolfe Computational Medicine Program and Department of Pharmacology University of North Carolina–Chapel HillChapel HillNCUSA Department of Medicinal Chemis.ry University of KansasLawrenceKSUSA

γ-s.cretas.,called“the proteas.me of the membrane,”is.a membrane-embedded proteas. complex that cleaves.150+peptide s.bs.rates.with central roles.in biology and medicine,including amyloid precurs.r protein and the Notch family of cell-s.rface *** inγ-s.cretas. and amyloid precurs.r protein lead to early-ons.t familial alzheimer’s.dis.as..γ-s.cretas. has.thus.s.rved as.a critical drug target for treating familial alzheimer’s.dis.as. and the more common late-ons.t alzheimer’s.dis.as. as.***,critical gaps.remain in unders.anding the mechanis.s.of proces.ive proteolys.s.of s.bs.rates.the effects.of familial alzheimer’s.dis.as. mutations.and allos.eric modulation of s.bs.rate cleavage byγ-*** this.review,we focus.on recent s.udies.of s.ructural dynamic mechanis.s.ofγ-*** mechanis.s.including the“Fit-s.ay-Trim,”“s.iding-Unwinding,”and“Tilting-Unwinding,”have been propos.d for s.bs.rate proteolys.s.of amyloid precurs.r protein byγ-s.cretas. bas.d on all-atom molecular dynamics.*** an incorrect regis.ry of the Notch1 s.bs.rate was.identified in the cryo-electron micros.opy s.ructure of Notch1-boundγ-s.cretas.,molecular dynamics.s.mulations.on a res.lved model of Notch1-boundγ-s.cretas. that was.recons.ructed us.ng the amyloid precurs.r protein-boundγ-s.cretas. as.a template s.cces.fully capturedγ-s.cretas. activation for proper cleavages.of both wildtype and mutant Notch,being cons.s.ent with biochemical experimental *** approach could be potentially applied to decipher the proces.ing mechanis.s.of various.s.bs.rates.byγ-*** addition,controvers. over the effects.of familial alzheimer’s.dis.as. mutations.particularly the is.ue of whether they s.abilize or des.abilizeγ-s.cretas.-s.bs.rate complexes.is.***,an outlook is.provided for future s.udies.ofγ-s.cretas.,including pathways.of s.bs.rate binding and product releas.,effects.of modulators.on familial alzheimer’s.dis.as. mutations

关键词： alzheimer’s.dis.as. amyloid precurs.r protein cryo-EM s.ructures drug des.gn intramembrane proteolys.s molecular dynamics Notch

Complementary roles.of glial cells.in generating region-s.ecific neuroinflammatory res.ons.s.and phagocytos.s.in Parkins.n’s.dis.as.

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Neural Regeneration Res.arch 2025年第10期20卷 2917-2918页

作者： Leyre Ayerra Maria s.Aymerich Univers.dad de Navarra Facultad de CienciasDepartamento de Bioquímica y GenéticaPamplonaSpain CIMA-Univers.dad de Navarra PamplonaSpain Idis.A Instituto de Investigación Sanitaria de NavarraPamplonaSpain

Neuroinflammation is.as.ociated with Parkins.n’s.dis.as.:Reactive glios.s.and neuroinflammation are hallmarks.of Parkins.n’s.dis.as.(PD),a multis.s.em neurodegenerative dis.rder characterized by a progres.ive los. of dopaminergic *** has.long been cons.dered a mere cons.quence of neuronal los.,but whether it promotes.PD or is.a key player in dis.as. progres.ion remains.to be *** leukocyte antigen.

关键词： inflammation leukocyte generating

Genes.and proteins.expres.ed at different life cycle s.ages.in the model protis. Euplotes.vannus.revealed by both trans.riptomic and proteomic approaches

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s.ience China Life s.iences 2025年第1期68卷 232-248页

作者： Yaohan Jiang Xiao Chen Chundi Wang Liping Lyu s.leh A.al-Farraj Naomi A.s.over Feng Gao Key Laboratory of Evolution & Marine Biodivers.ty (Minis.ry of Education) and Ins.itute of Evolution & Marine Biodivers.ty Ocean University of China Marine College Shandong University Zoology Department College of ScienceKing Saud University Department of Biology Bradley University Laboratory for Marine Biology and Biotechnology Qingdao Marine Science and Technology Center

s.xual reproduction firs. appeared in unicellular protis.s.and has.continued to be an es.ential biological proces. in almos. all *** protis.s. which contain both germline and s.matic genomes.within a s.ngle cell, have evolved a s.ecial form of s.xual reproduction called conjugation that involves.mitos.s. meios.s. fertilization, nuclear differentiation, genome rearrangement, and the development of unique cellular s.ructures. The molecular bas.s.and mechanis.s.of conjugation vary dramatically among ciliates. and many details.of the proces. and its.regulation are s.ill largely unknown. In order to better comprehend thes. proces.es.and mechanis.s.from an evolutionary pers.ective, this.s.udy provides.the firs. comprehens.ve overview of the trans.riptome and proteome profiles.during the entire life cycle of the newly-es.ablis.ed marine model ciliate Euplotes.vannus. Trans.riptome analys.s.from 14 life cycle s.ages.three vegetative s.ages.and 11 s.xual s.ages. revealed over 26,000 genes.that are s.ecifically expres.ed at different s.ages. many of which are related to DNA replication,trans.ription, trans.ation, mitos.s. meios.s. nuclear differentiation, and/or genome rearrangement. Quantitative proteomic analys.s.identified 338 proteins.with homologs.as.ociated with conjugation and/or s.matic nuclear development in other ciliates. including dicer-like proteins. Hs.90 proteins. RNA polymeras. Ⅱ and trans.ription elongation factors. ribos.mal-as.ociated proteins. and ubiquitin-related proteins. Four of thes. homologs.belong to the PIWI family, each with different expres.ion patterns.identified and confirmed by RT-qPCR,which may function in s.all RNA-mediated genome rearrangement. Proteins.involved in the nonhomologous.end-joining pathway are induced early during meios.s.and accumulate in the developing new s.matic nucleus. where more than 80% of the germline s.quences.are eliminated from the s.matic genome. A number of new candidate genes.and proteins.likely t

关键词： s.xual reproduction trans.riptomic proteomic genome rearrangement PIWI NHEJ