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Exome sequencing identifies a likely causative variant in 53%of families with ciliopathy-related features on renal ultrasound after excluding NPHP1 deletions

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Genes & Diseases 2024年第5期11卷 40-43页

作者： Konstantin Deutsch verena Klambt Thomas M.Kitzler Tilman Jobst-Schwan Ronen Schneider Florian Buerger Steve Seltzsam Sherif El Desoky Jameela A.Kari Farkhanda Hafeez maria Szczepanska Loai A.Eid Hazem S.Awad Muna Al-Saffar Neveen A.Soliman velibor Tasic Camille Nicolas-Frank Kirollos Yousef Luca M.Schierbaum Sophia Schneider Abdul Halawi Izzeldin Elmubarak Katharina Lemberg Shirlee Shril Shrikant M.Mane Nancy Rodig Friedhelm Hildebrandt Division of Nephrology Department of PediatricsBoston Children’s HospitalHarvard Medical SchoolBostonMA 02215USA Department of Pediatric Gastroenterology Nephrology and Metabolic DiseasesCharite Universitatsmedizin BerlinBerlin 13353Germany Berlin Institute of Health at Charitee Universitatsmedizin Berlin BIH Biomedical Innovation AcademyBIH Charite Clinician Scientist ProgramBerlin 10178Germany Department of Nephrology and Hypertension FriedrichAlexander-Universitat Erlangen-NurnbergErlangen 91054Germany Department of Pediatrics Faculty of Medicine King Abdulaziz UniversityPediatric Nephrology Center of ExcellenceKing Abdulaziz University HospitalJeddah 21589Saudi Arabia Department of Pediatric Nephrology The Children’s Hospital and Institute of Child HealthLahore 54000Pakistan Department of Pediatrics Faculty of Medical Sciences in ZabrzeMedical University of Silesia in KatowiceKatowice 40-752Poland Dubai Hospital and Al-Jalila Children’s Specialty Hospital Kidney Center of ExcellenceDubai 4545United Arab Emirates Department of Genetics and Genomics UAE UniversityAbu Dhabi 15551United Arab Emirates Department of Pediatrics Division of Genetics and GenomicsBoston Children’s HospitalHarvard Medical SchoolBostonMA 02215USA Department of Pediatrics Center of Pediatric Nephrology and TransplantationKasr Al Ainy School of MedicineCairo UniversityCairo 11562Egypt Egypt Center for Research and Regenerative Medicine(ECRRM) Cairo 11511Egypt Medical Faculty Skopje University Children’s HospitalSkopje 1000North Macedonia Department of Genetics Yale University School of MedicineNew HavenCT 06520USA

Nephronophthisis-related ciliopathies(NPHP-RC)represent one of the most common causes of chronic kidney disease in the first three decades of life and are characterized by a broad genetic and clinical heterogeneity.1 To date,more than 90 genes have been identified that cause autosomalrecessive NPHP-RC if mutated,accounting for up to 60%of cases.1 Among these,homozygous deletions of NPHP1 are the most common *** genes localize to primary cilia,basal bodies,or the centrosome and lead to a primary ciliary disruption if mutated,thereby causing a broad phenotypical spectrum.1 Patients that suffer from NPHP-RC can either have an isolated renal phenotype,such as polyuria,polydipsia,decreased urinary concentration ability,and secondary enuresis due to loss of tubular function,or present with extrarenal symptoms including retinal degeneration,cerebellar vermis hypoplasia,or hepatic *** are often diagnosed in early adolescence,reaching end-stage renal disease before 25 years of age,but early onset and rapidly progressive forms of NPHP-RC also exist.1 Renal ultrasound indicates kidneys of normal or reduced renal length with increased echogenicity and corticomedullary cysts.

关键词： degeneration urinary likely

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Evidence for polarization-induced phase transformations and degradation in CH_(3)NH_(3)PbI_(3)

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Nano Research 2023年第7期16卷 9435-9442页

作者： Aleksei Yu.Grishko maria A.Komkova Ekaterina I.Marchenko Alexandra v.Chumakova Alexey B.Tarasov Eugene A.Goodilin Andrei A.Eliseev Department of Materials Science MSU-BIT UniversityShenzhen 517182China Department of Chemistry Lomonosov Moscow State UniversityMoscow 119991Russia Department of Materials Science Lomonosov Moscow State UniversityMoscow 119991Russia European Synchrotron Radiation Facility F-38042 GrenobleFrance

In solar cells,hybrid halide perovskites operate under constant bias,thus their stability towards electric field-induced degradation is of key *** we report on evidence of previously unidentified electric field-induced transitions and degradation path of CH_(3)NH_(3)PbI_(3)(MAPbI_(3))using elemental and phase *** films of MAPbI_(3) were deposited onto 1-2μm-pitch interdigitated electrodes and subjected to direct current(DC)-*** MAPbI_(3) layer polarized withv/μm DC electric field undergoes pronounced ion redistribution to methylammonium-rich MAPbI_(3)-y(yvokes degradation of MAPbI_(3).Using nanofocus grazing-incidence wide-angle X-ray scattering(GIWAXS),we unambiguously showed that the bias voltage induces the transformation ofβ-MAPbI_(3) to metastableδ-MAPbI_(3) polymorph via alignment of polar organic cation with the electric *** transformation is partially reversible upon field ***,once formed,δ-MAPbI_(3) disrupts the morphology of pristine film and undergoes decomposition toβ-MAPbI_(3)(β-MAPI)and PbI_(2).With the aforementioned compositional and phase changes,only MA-rich part serves as the charge separation layer,while the I-rich excitation is blocked with the PbI_(2) barrier serving as holes trapping *** observations reveal the intermediate steps in electric-field-driven degradation of halide perovskites and show the role of polar cations in the process,which is instructive for further material design with higher stability metrics.

关键词： halide perovskites stability degradation electric field ionic conductivity stoichiometry

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Nitrosative stress in human spermatozoa causes cell death characterized by induction of mitochondrial permeability transition-driven necrosis

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Asian Journal of Andrology 2018年第6期20卷 600-607页

作者： Pamela Uribe maria E Cabrillana Miguel W Forn Favi/m Treulen Rodrigo Boguen vladimir Isachenko Evgenia Isachenko Ratil S inchez Juana v villegas Center of Reproductive Biotechnology-Scientific and Technological Bioresource Nucleus（CEBIOR-BIOREN） University of La FronteraTemuco 4811230Chile Center of Excellence in Translational Medicine University of La FronteraTemuco 4810296Chile Department of Internal Medicine Faculty of MedicineUniversity of La FronteraTemuco 4781218Chile %aboratory of Andrology Research of Mendoza（LIAM）Institute of Histology and Embriology of Mendoza（IHEM）Histology and Embryology Area Department of Morphology and PhysiologySchool of MedicineNational University of Cuyo and CCT-MendozaCONICETMendoza 5500Argentin SResearch Institute（CIUDA） Medicine FacultyAcencagua UniversityMendoza 5500Argentina Research Group for Reproductive Medicine Cologne UniversityCologne 50937Germany Department of Preclinical Sciences Faculty of MedicineUniversity of La FrenteraTemuco 4781218Chile.

Peroxynitrite is a highly reactive nitrogen species and a potent inducer of apoptosis and necrosis in somatic cells. Peroxynitrite-induced nitrosative stress has emerged as a major cause of impaired sperm function; however, its ability to trigger cell death has not been described in human spermatozoa. The objective here was to characterize biochemical and morphological features of cell death induced by peroxynitrite-mediated nitrosative stress in human spermatozoa. For this, spermatozoa were incubated with and without （untreated control） 3-morpholinosydnonimine （SIN-l）, in order to generate peroxynitrite. Sperm viability, mitochondrial permeability transition （MPT）, externalization of phosphatidylserine, DNA oxidation and fragmentation, caspase activation, tyrosine nitration, and sperm ultrastructure were analyzed. The results showed that at 24 h of incubation with SIN-l, the sperm viability was significantly reduced compared to untreated control （P〈 0.001）. Furthermore, the MPT was induced （P〈 0.01） and increment in DNA oxidation （P 〈 0.01）, DNA fragmentation （P 〈 0.01）, tyrosine nitration （P 〈 0.0001） and ultrastructural damage were observed when compared to untreated control. Caspase activation was not evidenced, and although phosphatidylserine externalization increased compared to untreated control （P 〈 0.001）, this process was observed in 〈10% of the cells and the gradual loss of viability was not characterized by an important increase in this parameter. In conclusion, peroxynitrite-mediated nitrosative stress induces the regulated variant of cell death known as MPT-driven necrosis in human spermatozoa. This study provides a new insight into the pathophysiology of nitrosative stress in human spermatozoa and opens up a new focus for developing specific therapeutic strategies to better preserve sperm viability or to avoid cell death.

关键词： necrosis nitrosative stress oxidative stress peroxynitrite sperm cell death

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Mechanical behavior of colonic anastomosis in experimental settings as a measure of wound repair and tissue integrity

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World Journal of Gastroenterology 2006年第35期12卷 5668-5673页

作者： Konstantinos A Ekmektzoglou Georgios C Zografos Stavros K Kourkoulis Ismene A Dontas Panagiotis K Giannopoulos Κaterina A Marinou maria v Poulakou Despina N Perrea Laboratory of Experimental Sur- gery and Surgical Research "NS Christeas" University of Athens Medical School Athens Greece 1st Univer- sity Department of General Surgery Athens School of Medicine Hippocration Hospital Athens Greece Laboratory of Testing and Materials De- partment of Mechanics National Technical University of Athens Greece BvMS MVM Laboratory of Experimen- tal Surgery and Surgical Research "NS Christeas" University of Athens Medical School Athens Greece and Greek Ministry of Rural Development and Food General Directorate of Veterinary Services

AIM: To determine the mechanical properties of anasto- motic colonic tissue in experimental settings and there- fore give a measure of wound healing. METHODS: Thirty-six male Wistar rats were used as ex- perimental models of anastomotic tissue integrity. On the 5th post-operative day, the tensile strength was measured by application of an axial force, providing a quantitative measure of anastomotic dehiscence and leakage. RESULTS: Diagrams of the load as a function of the time [P = P (t)] and of the displacement also as a func- tion of time [Δs = Δs (t)] were recorded for each test, permitting the design of the load versus the displace- ment diagram and thus providing significant data about the critical values of anastomotic failure. Quantitative data were obtained concerning the anastomotic strength of both control specimens (healthy rats), as well as spec- imens from non-healthy rats for comparison. CONCLUSION: This experimental model provides an excellent method of measuring anastomotic strength. Despite the relative small number of specimens used, this method provides an accurate way of measuring wound repair. More experimental measurements need to be performed to correlate emerging tensile strength val-ues to anastomotic failure.

关键词： Mechanical behavior Failure load Colon Anastomosis Wound healing

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Characterization of cerebral blood flow dynamics with multiscale entropy

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Journal of Innovative Optical Health Sciences 2017年第5期10卷 85-93页

作者： Alexey N.Pavlov Arkady S.Abdurashitov Olga N.Pavlova maria v.Ulanova Anastasia A.Bodrova Natalia A.Shushunova Alexander P.Khorovodov Oxana v.Semyachkina-Glushkovskaya Yuri Gagarin State Technical University of Saratov 77 Politechnicheskaya Street Saratov 410054Russia Saratov National Research State University 83 Astrakhanskaya Street Saratov 410012Russia

Based on the laser speckle contrast imaging(LSCI)and the multiscale entropy(MSE),we study in this work the blood flow dynamics at the levels of cerebral veins and the surrounding network of microcerebral *** discuss how the phenylephrine-related acute peripheral bypertension is refected in the cerebral circulation and show that the observed changes are scale dependent,and they are signifcantly more pronounced in microcerebral vessels,while the macrocerebral dynamics does not demonstrate authentic inter-group *** also consider the permeability of blood-brain barrier(BBB)and study its opening caused by sound *** show that alterations associated with the BBB opening can be revealed by the analysis of blood flow at the level of macrocerebral vessels.

关键词： Cerebral blood flow laser speckle contrast imaging arterial pressure complexity

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THE EXPERIMENTAL STUDY OF STRESS-RELATED PATHOLOGICAL CHANGES IN CEREBRAL vENOUS BLOOD FLOW IN NEWBORN RATS ASSESSED BY DOCT

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Journal of Innovative Optical Health Sciences 2013年第3期6卷 76-85页

作者： OXANA v.SEMYACHKINA-GLUSHKOvSKAYA vLADISLAv v.LYCHAGOv OLGA A.BIBIKOva IGOR A.SEMYACHKIN-GLUSHKOvSKIY SERGEY S.SINDEEv EKATERINAM.ZINCHENKO MOHHANAD M.KASSIM AL-FATLE FATEMA ALI AL HASSANI LEITH maria v.ULANOva vaLERY v.TUCHIN Department of Biology Saratov State UniversityAstrachanskaya Str.83410012 SaratovRussian Federation Department of Biology Al-Mustansiriya UniversityBaghdadIraq Institute of Precise Mechanics and Control of Russia Academy of Science Rabochaya 28 410028 SaratovRussian Federation Optoelectronics and Measurement Techniques Laboratory University of Oulu P.O.BOX 450090014 University of OuluFinland

In experiments on newbom rats with stress related intracrani al hemorhage(ICH)using Doppler optical coberence tomognaphy(DOCT)we have shown that latent stage of ICH(4 h after stress)is characterized by decrease of venous blood outflow and the loss of sensitivity of sagittal vein to vasoconstrictor ffect of *** incidence of ICH(24 h after stress)was accompanied by progressi on of early pathological changes in cerebral venous blood flow(CvBF)and development of venous *** into consideration of this fact,we suggest that the suppression of CvBF related to the severity to the deleterious effect of stress on the brain hemodynamics in newborn *** facts allow us to conclude that the venous insufficiency with the loss of vasoconstrictor response to adrenaline is an informative and sensitive component of pattern of CvBF that can be important diagnostic criteria of risk of ICH development in newborns.

关键词： Doppler optical coherence tomography stress related intracnanial hemorthage cerebral venous blood flow adrenaline

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Electrochemical studies on cerium(Ⅲ) in molten fluoride mixtures

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Journal of Rare Earths 2010年第3期28卷 428-434页

作者： vIRGIL CONSTANTIN ANA-maria POPESCU MIRCEA OLTEANU Romanian Academy Institute of Physical Chemistry "Ilie Murgulescu"Department of Molten Salts Splaiul Independentei 202Bucharest-Romania

This study aims to determine the principal electrochemical characteristics of the electrodeposition of cerium metal from molten fluoride *** cathodic process of Ce3+ ions in LiF-NaF and LiF-NaF-CaF2 molten salts was studied using electrochemical techniques as steady state and cyclic voltammetry *** decomposition potential(Ed) and the overvoltage(η) were determined for NaCeF4 using current-potential curves under galvanostatic *** Ed was found to be 2.025 v in LiF-NaF and 2.045 v in...

关键词： cerium molten salts lanthanides NaCeF4 electrolysis cyclic voltammetry

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Acromyrmex ameliae sp. n. （Hymenoptera： Formicidae）： A new social parasite of leaf-cutting ants in Brazil

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Insect Science 2007年第3期14卷 251-257页

作者： DANIvaL JOSE DE SOUZA ILKA maria FERNANDES SOARES TEREZINHA maria CASTRO DELLA LUCIA Institut de Recherche sur la Biologie de 1'Insecte Université Francois Rabelais Tours France Departamento de Biologia Animal Laboratório de Ecologia de Comunidades Departamento de Biologia Geral Universidade Federal de Vicosa MG 36570-000 Brazil

The fungus-growing ants （Tribe Attini） are a New World group of〉 200 species, all obligate symbionts with a fungus they use for food. Four attine taxa are known to be social parasites of other attines. Acromyrmex （ Pseudoatta） argentina argentina and Acromyrmex （Pseudoatta） argentina platensis （parasites of Acromyrmex lundi）, and Acromyrmex sp. （a parasite of Acromyrmex rugosus） produce no worker caste. In contrast, the recently discovered Acromyrmex insinuator （a parasite of Acromyrmex echinatior） does produce workers. Here, we describe a new species, Acromyrmex ameliae, a social parasite of Acromyrmex subterraneus subterraneus and Acromyrmex subterraneus brunneus in Minas Gerais, Brasil. Like A. insinuator, it produces workers and appears to be closely related to its hosts. Similar social parasites may be fairly common in the fungus-growing ants, but overlooked due to the close resemblance between parasite and host workers.

关键词： Acromyrmex leaf-cutting ants social evolution social parasitism

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Meso-substituted cationic 3-and 4-N-Pyridylporphyrins and their Zn(II)derivatives for antibacterial photodynamic therapy

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Journal of Innovative Optical Health Sciences 2022年第1期15卷 1-16页

作者： Aram G.Gyulkhandanyan Marina H.Paronyan Anna G.Gyulkhandanyan Karapet R.Ghazaryan Marina v.Parkhats Boris M.Dzhagarov maria v.Korchenova Ekaterina N.Lazareva Elena S.Tuchina Grigor v.Gyulkhandanyan valery v.Tuchin H.Buniatyan Institute of Biochemistry National Academy of Sciences of Armenia 5/1 Paruir Sevak Street Yerevan 0014Armenia INSERM U1204 Universite d'Evry vald'Essonne-Universite Paris-Saclay 1 Ruedu Pere JarlanBatiment Maupertuis91025Evry-CourcouronnesFrance B.I.Stepanov Institute of Physics National Academy of Sciences of Belarus 68-2 Nezavisimosti AvenueMinsk 220072Belarus Science Medical Center Saratov State University 83 Astrakhanskaya StreetSaratov 410012Russia Laboratory of Laser Molecular Imaging and Machine Learning(LMIML)National Research Tomsk State University 36 Lenin Avenue Tomsk 634050Russia Laboratory of Laser Diagnostics of Technical and Living Systems Institute of Precision Mechanics and Control Russian Academy of Sciences Saratov 410028Russia

Photodynamic inactivation of microorganisms known as antibacterial photodynamic therapy(APDT)is one of the most promising and innovative approaches for the destruction of pathogenic *** the photosensitizers(PSs),compounds based on cationic porphyrins/metalloporphyrins are most successfully used to inactivate *** of meso-substituted cationic pyridylporphyrins and metalloporphyrins with various peripheral groups in the third and fourth positions of the pyrrole ring have been synthesized in *** aim of this work was to determine and test the most e®ective cationic porphyrins and metalloporphyrins with high photoactivity against Gram negative and Gram positive *** was shown that the synthesized cationic pyridylporphyrins/metalloporphyrins exhibit a high degree of phototoxicity towards both types of bacteria,including the methicillinresistant *** *** complexes of porphyrins are more phototoxic than metal-free porphyrin *** e®ectiveness of these Zn–metalloporphyrins on bacteria is consistent with the level of singlet oxygen *** was found that the high antibacterial activity of the studied cationic porphyrins/metalloporphyrins depends on four factors:the presence in the porphyrin macrocycle of a positive charge(+4),a central metal atom(Zn2þÞand hydrophobic peripheral functional groups as well as high values of quantum yields of singlet *** results indicate that meso-substituted cationic pyridylporphyrins/metalloporphyrins cannd wider application in photoinactivation of bacteria than anionic or neutral PSs usually used in APDT.

关键词： Antibacterial photodynamic therapy cationic porphyrins/metalloporphyrins phototoxicity Zn-metalloporphyrins singlet oxygen quantum yield Gram negative and Gram positive bacteria S.aureus MRSA E.coli Salmonella typhimurium.

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Analgesic effect of inhaled lavender essential oil for frenotomy in healthy neonates:a randomized clinical trial

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World Journal of Pediatrics 2022年第6期18卷 398-403页

作者： Silvia Maya-Enero Montserrat Fàbregas-Mitjans Rosa maria Llufriu-Marquès Júlia Candel-Pau Jordi Garcia-Garcia MaríaÁngeles López-vílchez Department of Neonatology Service of PediatricsHospital del MarParc de Salut MarUniversitat Pompeu FabraPasseig Marítim 25-2908003BarcelonaSpain

Background Neonatal pain may affect long-term neurodevelopment and must be *** is a painful procedure wherein a common strategy to relieve pain(sucking)cannot be used because the technique is performed on the *** essential oil(LEO)has sedative and antispasmodic properties and has been successfully used to treat pain during heel puncture and *** aim was to demonstrate if the use of inhaled LEO is effective in reducing pain during frenotomy in healthy,full-term *** We conducted a randomized clinical trial in neonates who underwent a frenotomy between August 2020 and April *** assessed pain using pre and post-procedure heart rate and oxygen saturation,crying time and Neonatal Infant Pain Scale(NIPS)*** with type 3 tongue tie were randomized into the“experimental group”and“control group”.In both groups,we performed swaddling,administered oral sucrose,and let the newborn suck for two *** the experimental group,we also placed a gauze pad with one drop of LEO under the neonate’s nose for two minutes prior to and during the *** We enrolled 142 patients(71 per group).The experimental group showed significantly lower NIPS scores(1.88 vs 2.92)and cried almost half the amount of time(14.8 vs.24.6 seconds,P=0.006).Comparing with the control group,we observed no side effects in either of the *** We observed a significant decrease in crying time and lower NIPS scores in the neonates who received inhaled LEO and underwent a frenotomy for type 3 ***,we recommend using inhaled LEO during neonatal frenotomies.

关键词： Ankyloglossia Aromatherapy Frenotomy Lavender Neonate Neonatal pain Tongue-tie

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