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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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在密闭式的背后评LEGACY Signature SE落地式音箱[下篇]

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视听前线 2024年第10期 21-24页

作者： steve Feinstein 剑萍(编译) 不详

相关设备,系统的其余部分为:前置放大器/功率放大器组合为Parasound New Classic 2100/2250,在20-20kHz THD=0.05%的情况下额定功率保守地标示为200W(8 Q)/385W(4Q)/声道。SignatureSE为4Q音箱,轴上灵敏度为92dB1W/1m。不用说,Parasoun... 详细信息

关键词：落地式音箱前置放大器功率放大器解码芯片 Signature 密闭式额定功率能量驱动

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Confirmation of a Sub-Saturn-size Transiting Exoplanet Orbiting a G Dwarf:TOI-1194 b and a Very Low mass Companion Star: TOI-1251 B from TESS

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Research in Astronomy and Astrophysics 2024年第3期24卷 129-143页

作者： Jia-Qi Wang Xiao-Jun Jiang Jie Zheng Hanna Kellermann Arno Riffeser Liang Wang Karen A.Collins Allyson Bieryla Lars A.Buchhave steve B.Howell Elise Furlan Eric Girardin Joao Gregorio Eric Jensen Felipe murgas mesut Yilmaz Sam Quinn Xing Gao Ruo-Yu Zhou Frank Grupp Hui-Juan Wang CAS Key Laboratory of Optical Astronomy National Astronomical ObservatoriesChinese Academy Of SciencesBeijing 10010China University of Chinese Academy of Sciences Beijing 100049China University Observatory munich Scheinertrsse 1.D-81679 MunichGemany max Planck Institute for Extraterrestrial Physics Giessenbachstr.1D-85748 GarchingGermany National Astronomical Observatories/Nanjing Institute of Astronomical Optics&Technology Chinese Academy of SciencesNanjing210042China CAS Key Laboratory of Astronomical Optics&Technology Nanjing Institute of Astronomical Optics&TechnologyNanjing 210042China Center for Astrophysics Harvard&Smithsonian60 Garden Street.CambridgeMA 02138USA National Space Institute Technical University of DenmarkElektrovejDK-2800 KgsLyngbyDenmark NASA Ames Research Center Moffett FieldCA 94035USA NASA Exoplanet Science Institute Caltech/IPACMail Code 100-221200 E.California Blvd.PasadenaCA91125USA Grand Pra Observatory Switzerland Department of Physics Washington UniversitySt.LouisMO 63130.USA Department of Physics and Astronomy Swarthmore CollegeSwarthmorePA 19081USA Instituto de Astrofísica de Canarias(IAC) E-38205 La Laguna.TenerifeSpain Departamento de Astrofísica Universidad de La LagunaSpain Dept.of Astronomy and Space Sciences Ankara UniversityTandogan 06100AnkaraTurkey Xinjiang Astronomical Observatory Chinese Academy of SciencesUrumqi 830011China Urumqi No.1 Senior High School Urumqi 830002China Lijiang Gemini Observatory Lijiang 674199China

We report the confirmation of a sub-Saturn-size exoplanet,TOI-1194 b,with a mass of about 0.456+0.055-0.051m_(J),and a very low mass companion star with a mass of about 96.5±1.5 mJ,TOI-1251 *** candidates provided by the Transiting Exoplanet Survey Satellite(TESS)are suitable for further follow-up observations by ground-based telescopes with small and medium *** analysis is performed based on data from several telescopes worldwide,including telescopes in the sino-German multiband photometric campaign,which aimed at confirming TESS Objects of Interest(TOIs)using ground-based small-aperture and medium-aperture telescopes,especially for long-period ***-1194 b is confirmed based on the consistent periodic transit depths from the multiband photometric *** measure an orbital period of 2.310644±0.000001 days,the radius is 0.767+0.045-0.041RJ and the amplitude of the RV curve is 69.4_(-7.3)^(+7.9)m s^(-1).TOI-1251 B is confirmed based on the multiband photometric and high-resolution spectroscopic data,whose orbital period is 5.963054+0.000002-0.000001days,radius is 0.947+0.035-0.033 R_(J) and amplitude of the RV curve is 9849_(-40)^(+42)ms^(-1).

关键词： planets and satellites fundamental parameters-planets and satellites gaseous planets-stars fundamental parameters-stars low-mass-methods data analysis-techniques photometric-techniques spectroscopic

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Profile of Aggressive and Chronic Periodontitis in Kinshasa Dental Hospitals, DR Congo

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Open Journal of Stomatology 2017年第10期7卷 439-447页

作者： Em Kalala Kazadi steve sekele m.sin Nyimi Bushabu Fidele Isourady Bourley Jean Paul sekele Jacques Bolenge Augustin mantshumba Ingaya Ediz Ekofo mulumba Hubert Ntumba Unit of Oral Periodontics Surgery Stomatology Service Affiliated Hospital of Kinshasa University Kinshasa DR Congo Informatics and mathematics Department Faculty of Sciences Kinshasa University Kinshasa DR Congo Oral and maxillofacial Surgery Affiliated Hospital of Kinshasa University Kinshasa DR Congo Oral and maxillofacial Surgery School of Stomatology Wuhan University Wuhan China Prosthodontics and Orthodontics Service Affiliated Hospital of Kinshasa University Kinshasa DR Congo

Periodontitis is a prevalent destructive gum disease, characterized by loss of tissues supporting the teeth. The two main forms, chronic and aggressive have not been assessed in Kinshasa, DR Congo. Aim: The aim of this study was to investigate the Prevalence and the pattern of chronic periodontitis (CP) and aggressive periodontitis (AP) in order to improve its management. materials and methods: A cross sectional study of 404 subjects attending the dental services of the four hospitals of Kinshasa city, from October 2013 to April 2014 was carried out. The plaque index (PlI), bleeding on probing (BOP), probing pocket depth (PPD) and frequency of tooth brushing (FTB) were the parameters assessed. The SPSS software version 20.0 was used. Results: Out of 404 patients examined, 322 (79.7%) had chronic periodontitis versus to 44 (10.9%) of aggressive periodontitis. The female was higher than males with a sex ratio of 1.49:1 and the age group between 20 and 29 was the most represented. PPP of ≥6 mm was found in 24.6%, 5 or 5.5 mm in 27.3% and PPP of 3.5 to 4.5 mm in 48.1% of patients with periodontitis. Significant difference was found between CP and AP according to age variable, p-value main forms of periodontitis diseases are more prevalent in patients attending dental services in Kinshasa and affect male as well as female. Age remains an essential variable to distinguish between CP and AP.

关键词： Prevalence Profile Aggressive Periodontitis Chronic Periodontitis Probing Pocket Depth

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Determination of the Root Canal Length of Teeth of Bantu Patients Attending the Teaching Hospital of Kinshasa University

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Open Journal of Stomatology 2018年第1期8卷 16-23页

作者： Jean marie Kayembe Bukama Jean Paul sekele Issourdi Fidele Nyimi Bushabu Augustin mantshumba milolo steve sekele m.sin m. A. Agbor Dieudonne Nyembue Tshipukana Alain Nyengele Kayembe Hubert Ntumba mulumba Endodontic Unit Service of Operative Dentistry Teaching Hospital of Kinshasa University Kinshasa Democratic Republic of Congo Prosthodontics Services Teaching Hospital of Kinshasa University Kinshasa Democratic Republic of Congo Oral and maxillofacial Surgery Teaching Hospital of Kinshasa University Kinshasa Democratic Republic of Congo Department of Oral maxillofacial Head and Neck Oncology School and Hospital of Stomatology Wuhan University Wuhan China Department of mathematics and Computer Science Faculty of Science University of Kinshasa Kinshasa Democratic Republic of Congo Universite des montagne Dental School Bangangté Cameroon Department of Otorhinolaryngology (ENT) Teaching Hospital of Kinshasa University Kinshasa Democratic Republic of Congo

Background: There is paucity of literature on the determination of the root canal length of Bantu subjects in dental professional practicing in Africa and Democratic Republic of Congo in particular. Aims: The aim of the present study was to determine the root canal length of teeth of Bantu patients extracts attending the Teaching Hospital of Kinshasa University. methods and material: Prospective cross-sectional study was carried out in the service of Conservative Dentistry. The patients suffering with pulpitis of permanent teeth which were selected for root canal treatment during the period of January 2014 to December 2016 were included. All patients whose main root canals were inaccessible, teeth carrying prosthesis, teeth with large coronal decay, teeth having periapical periodontitis, supernumerary teeth, wisdom and primary teeth were excluded. Results: The upper canines presented some significant longer canals compared to the lower canine (23.4 ± 2.3 mm and 21.6 ± 1.8 mm). Palatal canals of the first and second molar were respectively longer as compared to the superior teeth canals (21.5 ± 1 mm, 21.3 ± 2 mm). The distal canals of the first and second molar were the longest in the mandibular arch respectively measuring 20.7 ± 2.0 mm and 21.5 ± 1.7 mm. Conclusion: Data obtained from Bantu patients show slightly shorter roots compared to some European populations, but longer than some Asian populations.

关键词： Bantu Length Root Canal X-Ray

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modern Black Sea oceanography applied to the end-Permian extinction event

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Journal of Palaeogeography 2015年第1期4卷 52-62页

作者： steve Kershaw Institute for the Environment Health and Societies Brunel UniversityKingston LaneUxbridge Middlesex UB8 3PH UK

The modern Black Sea has a mixed upper layer in the top 150-200 m of the water column, below which the water is anoxic, separated from the mixed layer by a redox boundary. There is limited vertical movement of water. Pyrite framboids form in the water column of the anoxic zone, then have been traditionally interpreted to sink immediately and accumulate in the sediments of the Black Sea. Thus the occurrence of framboids in sediments in the rock record is widely interpreted to indicate poorly oxygenated to anoxic conditions in ancient environments. However, in the Permian-Triassic boundary （PTB） microbialites of South China, which formed in shallow marine conditions in contact with the atmosphere, the published occurrence of framboids is inconsistent with abundant gastropod and ostracod shells in the microbialite. Furthermore, in the modern Black Sea, （a） framboids may be suspended, attached to organic matter in the water column, thus not settle to the sea floor immediately after formation; and （b） the redox zone is an unstable complex area subject to rapid vertical water movement including occasional upwelling. The model presented here supposes that upwelling through the redox zone can lead to upward transport of suspended pyrite framboids into the mixed layer. Advective circulation could then draw suspended framboids onto the shelf to be deposited in oxygenated sediments. In the Permian-Triassic transition, if framboids were upwelled from below the redox boundary and mixed with oxygenated waters, sediment deposited in these conditions could provide a mixed signal for potentially misleading interpretations of low oxygen conditions. However, stratigraphic sampling resolution of post-extinction microbialites is currently insufficient to demonstrate possible separation of framboid-bearing layers from those where framboids are absent. Profound differences between microbialite constructors and sequences between the western and eastern Tethys demonstrate barriers to migra

关键词： mass extinction Permian-Triassic boundary microbialite ostracods gastro-pods anoxia Black Sea

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Shift current bulk photovoltaic effect in polar materials—hybrid and oxide perovskites and beyond

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npj Computational materials 2016年第1期2卷 58-69页

作者： Liang Z Tan Fan Zheng steve m Young Fenggong Wang Shi Liu Andrew m Rappe The makineni Theoretical Laboratories Department of ChemistryUniversity of PennsylvaniaPhiladelphiaPAUSA Center for Computational materials Science United States Naval Research LaboratoryWashingtonDCUSA Geophysical Laboratory Carnegie Institution of ScienceWashingtonDCUSA

The bulk photovoltaic effect(BPVE)refers to the generation of a steady photocurrent and above-bandgap photovoltage in a single-phase homogeneous material lacking inversion *** mechanism of BPVE is decidedly different from the typical p-n junction-based photovoltaic mechanism in heterogeneous ***,there has been renewed interest in ferroelectric materials for solar energy conversion,inspired by the discovery of above-bandgap photovoltages in ferroelectrics,the invention of low bandgap ferroelectric materials and the rapidly improving power conversion efficiency of metal halide ***,as long as the nature of the BPVE and its dependence on composition and structure remain poorly understood,materials engineering and the realisation of its true potential will be *** this review article,we survey the history,development and recent progress in understanding the mechanisms of BPVE,with a focus on the shift current mechanism,an intrinsic BPVE that is universal to all materials lacking inversion *** addition to explaining the theory of shift current,materials design opportunities and challenges will be discussed for future applications of the BPVE.

关键词： mechanism effect ferroelectric

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FAK and Pyk2 in disease

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Frontiers in Biology 2016年第1期11卷 1-9页

作者： James m. murphy Hyeonsoo Park Ssang-Taek steve Lim Department of Biochemistry and molecular Biology College of Medicine University of South Alabama Mobile Alabama 36688 USA

Focal adhesion kinase （FAK） and proline-rich tyrosine kinase 2 （Pyk2） are non-receptor protein tyrosine kinases that are involved in cell proliferation, migration and survival. Current research of FAK and Pyk2 is greatly focused in cancer biology and several small molecule inhibitors are being tested under clinical development. Like cancer, certain chronic diseases such as cardiovascular disease, bone disease, fibrosis, rheumatoid arthritis, and neurological disorders, share malignant characteristics of cancer. Accumulating evidence has demonstrated that FAK and Pyk2 contribute to other proliferative and degenerative diseases. Thus, the goal of this review is to briefly highlight studies that have implicated FAK and Pyk2 as players in disease progression.

关键词： FAK Pyk2 FRNK disease

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Status of Oral Prosthetic Rehabilitation of Edentulism at the University Clinics of Kinshasa (CUK), DR Congo

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Open Journal of Stomatology 2021年第6期11卷 244-249页

作者： I. B. J. P. sekele m. K. H. Ntumba P. S. J. Lutula m. N. P. sekele B. F. Nymi Prosthodontics Service Department of Dental Medicine Faculty of Medicine Kinshasa University Kinshasa Democratic Republic of Congo National Center Laboratory of Oral Biomedicine ministry of Research Innovation and Technology Kinshasa Democratic Republic of Congo Oral and maxillofacial Surgery Service Department of Dental Medicine Faculty of Medicine Kinshasa University Kinshasa Democratic Republic of Congo

Objective: The present work aimed to identify the most common edentulousness and prosthesis type (prosthetic treatment), to improve the management in oral prosthetic rehabilitation in DR Congo. material and methods: It was a documentary, longitudinal, and retrospective study of the medical records of edentulous patients admitted to the prosthetic service of the Dental Department/Kinshasa University from January 1983 to December 2020. Age, sex, cause of teeth loss, and prosthetic treatment (partial removable prosthesis, complete removable prosthesis) were evaluated. The Chare square test was performed to compare significant differences between the variables and the P-value Results: One thousand six hundred and ninety patients in that 901 were men (47 ± 16 years) and 789 women (42 ± 15 years) had undergone prosthetic treatment. One thousand eight hundred and forty-four edentulous teeth were viewed according to the Kennedy classification. Kennedy class 1 was the most predominant (61.4%) followed by Kennedy class 3 (24.8%). Two thousand one hundred and ninety-one prostheses were performed. The removable partial prosthesis with plate (acrylic resin) was the most performed (78.8%;n = 1727) and followed by the joint (17.4%;n = 384). Dental caries (52.6%) and periodontitis (36.4%) were the main causes of these edentulous teeth. Conclusion: The present study showed that edentulism is becoming a concern for the implementation of a real oral health policy.

关键词： Prosthetic Rehabilitation Edentulousness

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Facile synthesis of insulin fusion derivatives through sortase A ligation

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Acta Pharmaceutica sinica B 2021年第9期11卷 2719-2725页

作者： maria m.Disotuar Jake A.Smith Jinze Li steve Alam Nai-Pin Lin Danny Hung-Chieh Chou Department of Biochemistry School of MedicineUniversity of UtahSalt Lake CityUT 84112USA Division of Endocrinology and Diabetes Department of PediatricsSchool of MedicineStanford UniversityPalo AltoCA 94305USA

Insulin derivatives such as insulin detemir and insulin degludec are *** and Drug Administration(FDA)-approved long-acting insulin currently used by millions of people with *** derivatives are modified in C-terminal B29 lysine to retain insulin *** and efficient methods for facile synthesis of insulin derivatives may lead to new discovery of therapeutic ***,we report a new method using sortase A(SrtA)-mediated ligation for the synthesis of insulin derivatives with high efficiency and functional group tolerance in the C-terminal B *** new insulin molecule(Ins-SA) with an SrtA-recognizing motif can be conjugated to diverse groups with N-terminal oligoglycines to generate new insulin *** further demonstrated that a new insulin derivative synthesized by this SrtA-mediated ligation shows strong cellular and in vivo *** enzymatic method can therefore be used for future insulin design and development.

关键词： Diabetes mellitus Insulin synthesis Sortase A(SrtA)ligation Albumin-binding peptide SA21 Long-acting insulin

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