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Progressive familial intrahepatic cholestasis—farnesoid X receptor deficiency due to NR1H4 mutation:A case report

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World Journal of Clinical Cases 2021年第15期9卷 3631-3636页

作者： Piotr Czubkowski Richard J Thompson Irena Jankowska A S Knisely Milton Finegold Pamela Parsons joanna Cielecka-Kuszyk Sandra Strautnieks joanna Pawłowska Laura N Bull Department of Gastroenterology HepatologyNutritional Disorders and PediatricsThe Children’s Memorial Health InstituteWarsaw 04-730Poland Institute of Liver Studies King's College London HospitalLondon SE59RSUnited Kingdom Institut für Pathologie Medizinische Universität GrazGraz 8010Austria Department of Pathology and Immunology Baylor College of MedicineHoustonTX 77030United States Texas Children’s Hospital HoustonTX 77030United States Department of Pathology The Children’s Memorial Health InstituteWarsaw 04-730Poland Department of Medicine and Institute for Human Genetics UCSF Liver Center LaboratoryUniversity of California San FranciscoSan FranciscoCA 94143United States

BACKGROUND Functioning farnesoid X receptor(FXR;encoded by NR1H4)is key to normal bile acid *** mutations in NR1H4 are reported in a few children with intrahepatic *** describe a boy with progressive familial intrahepatic cholestasis and homozygous mutation in *** SUMMARY A boy had severe neonatal cholestasis with moderate hypercholanemia and persistently elevated *** medical treatment,coagulopathy was uncontrollable,prompting liver transplantation at age 8 mo with incidental *** patient experienced catch-up growth with good liver function and did not develop allograft ***,1 year after transplant,he died from an acute infection,considered secondary to immunosuppression and asplenia.A homozygous protein-truncating mutation,c.547C>T,p.(Arg183Ter),was subsequently identified in NR1H4,and both parents were shown to be heterozygous *** of FXR and of bile salt export pump expression was confirmed by immunostaining of explanted *** Severe cholestasis with persistently high alpha-fetoprotein and modest elevation of serum bile acid levels may suggest FXR *** patients with FXR deficiency may not develop allograft steatosis and may respond well to liver transplantation.

关键词： Neonatal cholestasis Progressive familial intrahepatic cholestasis Bile salt export pump Liver transplantation Alpha-fetoprotein Case report

Impacts of common factors of life style on serum liver enzymes

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World Journal of Gastroenterology 2014年第33期20卷 11743-11752页

作者： joanna Danielsson Pivikki Kangastupa Tiina Laatikainen Mauri Aalto Onni Niemel Department of Laboratory Medicine Sein?joki Central Hospital and University of Tampere 60220 Sein?joki Finland Medical Research Unit Sein?joki Central Hospital and University of Tampere 60220 Sein?joki Finland National Institute for Health and Welfare (THL) 00271 Helsinki Finland The Institute of Public Health and Clinical Nutrition University of Eastern Finland 70211 Kuopio Finland Hospital District of North Karelia 80210 Joensuu Finland

AIM: To investigate the impacts of gender, age and factors of life style (alcohol, overweight, coffee and smoking) on serum liver enzymes.

关键词： Alcohol Obesity Aging Smoking Liver en-zymes Oxidative stress

Effect of the C:N:P ratio on the denitrifying dephosphatation in a sequencing batch biofilm reactor(SBBR)

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Journal of Environmental Sciences 2015年第12期27卷 119-125页

作者： Artur Mielcarek joanna Rodziewicz Wojciech Janczukowicz Arthur J.Thornton Tomasz Józwiak Paula Szymczyk University of Warmia and Mazury in Olsztyn Department of Environment Engineering ul. Warszawska 117a Olsztyn 10-719 Poland. Atkins Woodcote Grove Ashley Road Epsom KT18 United Kingdom

A series of investigations were conducted using sequencing batch biofilm reactor（SBBR） to explore the influence of C：N：P ratio on biological dephosphatation including the denitrifying dephosphatation and the denitrification *** in the reactor occurred mainly in the form of a biofilm attached to completely submerged *** acid was used as the source of organic carbon.C：N：P ratios have had a significant effect on the profiles of phosphate release and phosphate uptake and nitrogen *** highest rates of phosphate release and phosphate uptake were recorded at the C：N：P ratio of 140：70：*** C：N ratio of 2.5：1 ensured complete *** highest rate of denitrification was achieved at the C：N：P ratio of 140：35：*** increase of nitrogen load caused an increase in phosphates removal until a ratio C：N：P of 140：140：*** of the biofilm exposed to alternate conditions of mixing and aeration exhibited enhanced intracellular accumulation of ***,the structure of the biofilm encouraged anaerobic-aerobic as well as anoxic-anaerobic and absolutely anaerobic conditions in a *** heterogeneous conditions in the presence of nitrates may be a significant factor determining the promotion of denitrifying polyphosphate accumulating organism（DNPAO） development.

关键词： Wastewater treatment Sequencing batch biofilm reactor Nitrogen and phosphorus removal Enhanced intracellular polyphosphate accumulation Denitrifying dephosphatation Denitrification and dephosphatation rates

Hepatitis C in human immunodeficiency virus co-infected individuals: Is this still a “special population”?

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World Journal of Hepatology 2015年第15期7卷 1936-1952页

作者： Drosos E Karageorgopoulos joanna Allen Sanjay Bhagani Department of Infectious Diseases/HIV Medicine Royal Free London NHS Foundation Trust Research Department of Infection UCL Medical School Royal Free Hospital

A substantial proportion of individuals with chronic hepatitis C virus(HCV) are co-infected with human immunodeficiency virus(HIV). Co-infected individuals are traditionally considered as one of the "special populations" amongst those with chronic HCV, mainly because of faster progression to end-stage liver disease and suboptimal responses to treatment with pegylated interferon alpha and ribavirin, the benefits of which are often outweighed by toxicity. The advent of the newer direct acting antivirals(DAAs) has given hope that the majority of co-infected individuals can clear HCV. However the "special population" designation may prove an obstacle for those with co-infection to gain access to the new agents, in terms of requirement for separate pre-licensing clinical trials and extensive drug-drug interaction studies. We review the global epidemiology, natural history and pathogenesis of chronic hepatitis C in HIV co-infection. The accelerated course of chronic hepatitis C in HIV co-infection is not adequately offset by successful combination antiretroviral therapy. We also review the treatment trials of chronic hepatitis C in HIV co-infected individuals with DAAs and compare them to trials in the HCV mono-infected. There is convincing evidence that HIV co-infection no longer diminishes the response to treatment against HCV in the new era of DAA-based therapy. The management of HCV co-infection should therefore become a priority in the care of HIV infected individuals, along with public health efforts to prevent new HCV infections, focusing particularly on specific patient groups at risk, such as men who have sex with men and injecting drug users.

关键词： Human immunodeficiency virus HepatitisC Coinfection Antiviral agents Anti-retroviral agents Natural history Epidemiology Pathogenesis Therapy

Reflection on the Challenges,Accomplishments,and New Frontiers of Gene Drives

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BioDesign Research 2022年第1期2022卷 177-192页

作者： Michael Melesse Vergara Jesse Labbé joanna Tannous Biosciences Division Oak Ridge National LaboratoryOak RidgeTN 37831USA Invaio Sciences CambridgeMA 02138USA

Ongoing pest and disease outbreaks pose a serious threat to human, crop, and animal lives, emphasizing the need for constantgenetic discoveries that could serve as mitigation strategies. Gene drives are genetic engineering approaches discovered decadesago that may allow quick, super-Mendelian dissemination of genetic modifications in wild populations, offering hopes formedicine, agriculture, and ecology in combating diseases. Following its first discovery, several naturally occurring selfishgenetic elements were identified and several gene drive mechanisms that could attain relatively high threshold populationreplacement have been proposed. This review provides a comprehensive overview of the recent advances in gene drive researchwith a particular emphasis on CRISPR-Cas gene drives, the technology that has revolutionized the process of genomeengineering. Herein, we discuss the benefits and caveats of this technology and place it within the context of natural genedrives discovered to date and various synthetic drives engineered. Later, we elaborate on the strategies for designing syntheticdrive systems to address resistance issues and prevent them from altering the entire wild populations. Lastly, we highlight themajor applications of synthetic CRISPR-based gene drives in different living organisms, including plants, animals, andmicroorganisms.

关键词： offering benefits advances

Toxicity studies of elemental sulfur in marine sediments

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International Journal of Sediment Research 2018年第2期33卷 191-197页

作者： Monika Cieszynska-Semenowicz Justyna Rogowska Wojciech Ratajczyk joanna Ratajczyk Lidia Wolska Department of Environmental Toxicology Faculty of Health Sciences Medical University of Gdansk

Elemental sulfur(ES) is a component essential for proper development of animals, but it can be toxic for aquatic organisms. The objectives of the study reported here included determination of ES concentrations in sediment collected in the area of the Gulf of Gdansk(Baltic Sea) and search for the possible correlations with the ecotoxicity results. Sediment samples were collected from four locations: the Vistula River mouth, in the Port Basin, in the area of discharge of the treated wastewater from the Wastewater Treatment Plant(WWTP) and in the area of the sunken World War Ⅱ s/s 'Stuttgart' shipwreck. The levels of elemental sulfur were determined using a gas chromatograph coupled with a mass spectrometer(GC-MS). Toxicity of marine sediment samples was estimated for three biotest organisms-bioluminescent bacteria Vibrio fischeri, crustacean Heterocypris incongruens, and Synapis alba plant. The highest toxicity of sediments for all indicator organisms was observed in samples taken at the place of the shipwreck(2-100%). The same samples had a high sulfur content(16.7-143.2 μg/g dry weight(d.w.))therefore, it was decided to investigate whether the presence of ES in the sediment in the studied area can have an impact on the results of the ecotoxicity determination in real samples. However, the removal of sulfur from the samples resulted in no significant changes in the level of toxicity of the samples. In this research it could not be confirmed that the presence of elemental sulfur is the only factor responsible for the observed sediment toxicity. It seems that other compounds or their mixtures present in the sediment may have a significant influence on the results obtained.

关键词： Elemental sulfur(ES) Ecotoxicity Sediment Aquatic environment

Molecular dynamics-driven exploration of peptides targeting SARS-CoV-2,with special attention on ACE2,S protein,M^(pro),and PL^(pro):A review

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BIOCELL 2023年第8期47卷 1727-1742页

作者： MOHAMAD ZULKEFLEE SABRI joanna BOJARska FAI-CHU WONG TSUN-THAI CHAI Green Chemistry and Sustainable Technology Cluster Bioengineering SectionUniversiti Kuala LumpurMalaysian Institute of Chemical and Bioengineering Technology(UniKL MICET)Alor Gajah78000Malaysia Institute of General and Ecological Chemistry Faculty of ChemistryLodz University of TechnologyLodz90-924Poland Department of Chemical Science Faculty of ScienceUniversiti Tunku Abdul RahmanKampar31900Malaysia Center for Agriculture and Food Research Universiti Tunku Abdul RahmanKampar31900Malaysia

Molecular dynamics(MD)simulation is a computational technique that analyzes the movement of a system of particles over a given *** can provide detailed information about the fluctuations and conformational changes of biomolecules at the atomic level over *** recent years,MD has been widely applied to the discovery of peptides and peptide-like molecules that may serve as severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)*** review summarizes recent advances in such explorations,focusing on four protein targets:angiotensin-converting enzyme 2(ACE2),spike protein(S protein),main protease(M^(pro)),and papain-like protease(PL^(pro)).These four proteins are important druggable targets of SARS-CoV-2 because of their roles in viral entry,maturation,and infectivity of the virus.A review of the literature revealed that ACE2,S protein,and M^(pro) have received more attention in MD research than PL^(pro).Inhibitors of the four targets identified by MD simulations included peptides derived from food and other bioresources,peptides designed using the targets as templates,and peptide-like molecules retrieved from *** of the inhibitors have yet to be validated in experimental assays for ***,the role of MD simulation as an efficient tool in the early stages of anti-SARS-CoV-2 drug discovery agents has been demonstrated.

关键词： COVID-19 Anti-SARS-CoV-2 agent Receptor binding domain Computational screening Drug discovery

Population differentiation at a regional scale in spadefoot toads： contributions of distance and divergent selective environments

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Current Zoology 2016年第2期62卷 193-206页

作者： Amber M. RICE Michael A. MCQUILLAN Heidi A. SEEARS joanna A. WARREN Department of Biological Sciences Lehigh University BethLehem PA 18015 USA

The causes of population differentiation can provide insight into the origins of early barriers to gene flow. Two key drivers of population differentiation are geographic distance and local adaptation to divergent selective environments. When reproductive isolation arises because some populations of a species are under selection to avoid hybridization while others are not, population differentiation and even speciation can result. Spadefoot toad populations Spea multiplicata that are sympatric with a congener have undergone reinforcement. This reinforcement has resulted not only in increased reproductive isolation from the congener, but also in the evolution of reproductive isolation from nearby and distant conspecific allopatric populations. We used multiple approaches to evaluate the contributions of geographic distance and divergent selective environments to population structure across this regional scale in S. multiplicata, based on genotypes from six nuclear microsatellite markers. We compared groups of populations varying in both geographic location and in the presence of a congener. Hierarchical F-statistics and results from cluster analyses and discriminant analyses of principal components all indicate that geographic distance is the stronger contributor to genetic differentiation among S. multiplicata populations at a regional scale. However, we found evidence that adaptation to divergent selective environments also contributes to population structure. Our findings highlight how variation in the balance of evolutionary forces acting across a species＇ range can lead to variation in the relative contributions of geographic distance and local adaptation to population differentiation across different spatial scales.

关键词： cascade reinforcement character displacement reproductive isolation spatial scale Spea multipficata speciation

An Involution over Dyck Paths Related with Stirling Statistics

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Journal of Mathematical Research with Applications 2024年第4期44卷 437-443页

作者： Peng CAO joanna N.CHEN College of Science Tianjin University of Technology

We construct an involution over Dyck paths, which implies the distribution of statistics “the number of peak”, “the number of returns” and “the height of the last peak”. As an application, equidistributions of s... 详细信息

关键词： involutions Dyck paths pattern peak returns