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Lentiviral Gene Therapy for Artemis-Deficient ScID

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四川生理科学杂志 2022年第12期44卷 2107-2107页

作者： morton j cowan from the departments of pediatrics (m.j.c. J.Y. J.F. C.F.-B. U.S. M.K. J.D. J.L.-B. W.C. S.C. R.C. C.C.D. J.M.P.) and Epidemiology and Biostatistics (J.F.H.) the Smith Cardiovascular Research Institute (M.J.C. J.M.P.) and the School of Pharmacy (J.L.-B.) University of California San Francisco (UCSF) and UCSF Benioff Children’s Hospital (M.J.C. J.F. J.D. J.L.-B. J.O. C.C.D. J.M.P.) San Francisco the Department of Pediatrics University of California San Diego and Rady Children’s Hospital San Diego (L.B.) and the Department of Pediatrics UCLA Mattel Children’s Hospital Los Angeles (C.Y.K.) — all in California the Department of pediatrics Johns Hopkins All Children’s Hospital St. Petersburg FL (D.C.) the Department of pediatrics Sainte-Justine University Hospital Center University of Montreal Montreal (H.D.) Tuba city Regional Health care Tuba City (C.G. D.H.) and Phoenix Children’s Hospital Phoenix (H.K.M.) — both in Arizona the Department of pediatrics University of Washington Seattle Children’s Hospital Seattle (A.P.) clinical Development Roche Diagnostics Solutions Singapore (D.P.) the National Institute of Allergy and Infectious Diseases National Institutes of Health Bethesda MD (H.L.M.) and the Department of Genetics Cell Biology and Development University of Minnesota Minneapolis (R.S.M.).

Background:The DNA-repair enzyme Artemis is essential for rearrangement of T-and B-cell *** in DcLRE1c,which encodes Artemis,cause Artemis-deficient severe combined immunodeficiency(ART-ScID),which is poorly responsiv... 详细信息

关键词： ScID immunodeficiency Artemis

Inflammasome links traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease

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Neural Regeneration Research 2025年第6期20卷 1644-1664页

作者： Gabriela Seplovich Yazan Bouchi juan Pablo de Rivero Vaccari jennifer c.munoz Pareja Andrew Reisner Laura Blackwell Yehia mechref Kevin K.Wang j.Adrian Tyndall Binu Tharakan Firas Kobeissy Department of Surgery Morehouse School of MedicineAtlantaGAUSA Department of Neurobiology Center for NeurotraumaMultiomics&Biomarkers(CNMB)Neuroscience InstituteMorehouse School of MedicineAtlantaGAUSA Department of Neurological Surgery and the miami Project to cure Paralysis University of Miami Miller School of MedicineMiamiFLUSA Division of Pediatric critical care Department of PediatricsUniversity of Miami Miller School of MedicineMiamiFLUSA Department of pediatrics Emory UniversityAtlantaGAUSA Department of Neurosurgery Children’s Healthcare of AtlantaAtlantaGAUSA Department of chemistry and Biochemistry Texas Tech UniversityLubbockTXUSA morehouse School of medicine AtlantaGAUSA

Traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease are three distinct neurological disorders that share common pathophysiological mechanisms involving neuroinflammation. One sequela of neuroinflammation includes the pathologic hyperphosphorylation of tau protein, an endogenous microtubule-associated protein that protects the integrity of neuronal cytoskeletons. Tau hyperphosphorylation results in protein misfolding and subsequent accumulation of tau tangles forming neurotoxic aggregates. These misfolded proteins are characteristic of traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease and can lead to downstream neuroinflammatory processes, including assembly and activation of the inflammasome complex. Inflammasomes refer to a family of multimeric protein units that, upon activation, release a cascade of signaling molecules resulting in caspase-induced cell death and inflammation mediated by the release of interleukin-1β cytokine. One specific inflammasome, the NOD-like receptor protein 3, has been proposed to be a key regulator of tau phosphorylation where it has been shown that prolonged NOD-like receptor protein 3 activation acts as a causal factor in pathological tau accumulation and spreading. This review begins by describing the epidemiology and pathophysiology of traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease. Next, we highlight neuroinflammation as an overriding theme and discuss the role of the NOD-like receptor protein 3 inflammasome in the formation of tau deposits and how such tauopathic entities spread throughout the brain. We then propose a novel framework linking traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease as inflammasomedependent pathologies that exist along a temporal continuum. Finally, we discuss potential therapeutic targets that may intercept this pathway and ultimately minimize long-term neurological decline.

关键词： Alzheimer's disease caspase-1 chronic traumatic encephalopathy inflammasomes neurodegeneration neuroinflammation NLRP1 NLRP3 pyroptosis tauopathy traumatic brain injury

cysticercosis of the neck:a case report

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Asian Pacific journal of Tropical medicine 2010年第10期3卷 833-834页

作者： chandrakant Patil Rashmi Kharat(Patil) Prasad Deshmukh Blenda D'Souza Department of E.N.T J.N.M.CSawangiWardhaMaharashtra

The neck is a rare site for *** few cases of cysticercosis presenting in the neck have been *** report one such case creating diagnostic dilemma.

关键词： cysticercosis Neck

Acute hemorrhagic edema of infancy: the experience of a large tertiary pediatric center in Israel

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World journal of pediatrics 2017年第4期13卷 341-345页

作者： Limor Parker Keren Shahar-Nissan Liat Ashkenazi-Hoffnung Liora Harel jacob Amir Omer Trivizki Efraim Bilavsky Department of pediatrics B Assaf Harofeh Medical CenterZriffinIsrael departments of pediatrics c Schneider Children's Medical CenterPetah TiqvaIsrael Sackler Faculty of medicine Tel Aviv UniversityTel AvivIsrael

Background:Acute hemorrhagic edema of infancy (AHEI) is a rare leukocytoclastic vasculitis of the small vessels occurring at a young age and considered as a benign self-limited *** to its low prevalence,there are limited data on the presentation and complications of this ***:All computerized files of children who were hospitalized at a tertiary pediatric center due to AHEI over a 10 year period were ***,laboratory and histopathological data were ***:Twenty-six patients were included in our study,accounting for 0.7 cases per 1000 admissions of children aged 2 years or *** age was 12.9 *** than two thirds of the children had preceding symptoms compatible with a viral *** admission,all patients presented with typical findings of a rash and *** was most profound over the lower extremities (73%).concomitant viral or bacterial infections were found in six *** biopsy was performed in six patients revealing leukocytoclastic *** children (50%) had systemic involvement including joint involvement (n=9),gastrointestinal hemorrhage (n=4),microscopic hematuria (n=1) and compartment syndrome of the limb (n=1).The latter was diagnosed in a patient with familial mediterranean ***:Our largest data series highlighted what is known regarding clinical and histological findings in children with ***,contrary to what was previously reported,we found a higher rate of systemic *** AHEI is a rare entity,pediatricians should be familiar with its presentation,management and our reported complications.

关键词： acute hemorrhagic edema Finkelstein-Seidlmayer disease Henoch-Sch(o)nlein purpura vasculitis

A time for cocktails and inclusion

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Neural Regeneration Research 2018年第6期13卷 987-988页

作者： Shenandoah Robinson Frances j.Northington Lauren L.jantzie Division of Pediatric Neurosurgery Johns Hopkins University School of Medicine Division of Neonatology Department of Pediatrics Johns Hopkins University School of Medicine departments of pediatrics and Neurosciences University of New Mexico School of Medicine

Perinatal brain injury（PBI）is one of the most important causes of lifelong deficits in cognition,behavior,social interaction and motor skills,as well as *** encompasses,but is not limited to,hypoxic-ischemic encepha... 详细信息

关键词： PBI www HIE EPO A time for cocktails and inclusion

Optimizing stem cells for cardiac repair:current status and new frontiers in regenerative cardiology

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World journal of Stem cells 2017年第1期9卷 9-25页

作者： Shant Der Sarkissian Thierry Lévesque Nicolas Noiseux Department of Surgery Faculté de médecine Université de MontréalMontrealQC H3C 3J7Canada Facultéde médecine Universitéde MontréalMontrealQC H3C 3J7Canada

cell therapy has the potential to improve healing of ischemic heart, repopulate injured myocardium and restore cardiac function. The tremendous hope and potential of stem cell therapy is well understood, yet recent trials involving cell therapy for cardiovascular diseases have yielded mixed results with inconsistent data thereby readdressing controversies and unresolved questions regarding stem cell efficacy for ischemic cardiac disease treatment. These controversies are believed to arise by the lack of uniformity of the clinical trial methodologies, uncertainty regarding the underlying reparative mechanisms of stem cells, questions concerning the most appropriate cell population to use, the proper delivery method and timing in relation to the moment of infarction, as well as the poor stem cell survival and engraftment especially in a diseased microenvironment which is collectively acknowledged as a major hindrance to any form of cell therapy. Indeed, the microenvironment of the failing heart exhibits pathological hypoxic, oxidative and inflammatory stressors impairing the survival of transplanted cells. Therefore, in order to observe any significant therapeutic benefit there is a need to increase resilience of stem cells to death in the transplant microenvironment while preserving or better yet improving their reparative functionality. Although stem cell differentiation into cardiomyocytes has been observed in some instance, the prevailing reparative benefits are afforded through paracrine mechanisms that promote angiogenesis, cell survival, transdifferentiate host cells and modulate immune responses. Therefore, to maximize their reparative functionality, ex vivo manipulation of stem cells through physical, genetic and pharmacological means have shown promise to enable cells to thrive in the postischemic transplant microenvironment. In the present work, we will overview the current status of stem cell therapy for ischemic heart disease, discuss the most recurring c

关键词： Stem cell Regenerative medicine cellular cardiomyoplasty Preconditioning myocardial infarction Heart failure Viability Paracrine activity Transplantation Pharmaco-optimizer

management of glaucoma in pregnancy:risks or choices,a dilemma?

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International journal of Ophthalmology(English edition) 2016年第11期9卷 1684-1690页

作者： Harinder Singh Sethi mayuresh Naik Vishnu Swarup Gupta Department of Ophthalmology V.M.M.C & Safdarjung Hospital

The treatment of glaucoma in and around pregnancy offers the unique challenge of balancing the risk of vision loss to the mother as against the potential harm to the fetus or newborn. most anti-glaucoma drugs（***-blockers, prostaglandin analogues, carbonic anhydrase inhibitors topical and systemic, cholinergics,anticholinesterases, and apraclonidine） are considered category c agents and ophthalmologists are usually limited to treating patients with the category B drugs of brimonidine and dipivefrin. Brimonidine is generally the preferred first-line drug in the first, second and early third trimester. Late in the third trimester, brimonidine should be discontinued because it can induce central nervous system depression in newborns wherein topical carbonic anhydrase inhibitors may be the optimal *** surgery can be performed with caution in second and third trimester if the patients have a strong indication for the procedure. However, anesthetics,sedative agents, and antimetabolites still have potential risk for the fetus. Argon laser trabeculoplasty（ALT） or selective laser trabeculoplasty（SLT） is an alternative treatment that can be performed in all *** anhydrase inhibitors and β-blockers are certified by the American Academy of pediatrics for use during nursing. However, low doses of these medications should be considered when used in the breast feeding period. Optimum treatment for glaucoma in pregnancy must not be withheld so as to prevent any further deterioration in progressive vision loss and quality of life.

关键词： glaucoma pregnancy brimonidine argonlaser trabeculoplasty-selective laser trabeculoplasty

Seismic reflection and transmission coefficients of a single layer sandwiched between two dissimilar poroelastic solids

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Petroleum Science 2017年第4期14卷 676-693页

作者： manjeet Kumari mahabir Singh Barak manjeet Kumar Department of mathematics Indira Gandhi UniversityMeerpurRewari-122505India Department of mathematics C.M.R.J Govt.CollegeEllenabadSirsa-125102India

The seismic reflection and transmission characteristics of a single layer sandwiched between two dissimilar poroelastic solids saturated with two immiscible viscous fluids are investigated. The sandwiched layer is modeled as a porous solid with finite thickness. The propagation of waves is represented with potential functions. The displacements of particles in different phases of the aggregate are defined in terms of these potential functions. Due to the presence of viscosity in pore fluids, the reflected and transmitted waves are inhomogeneous in nature, i.e., with different directions of propagation and attenuation. The closed-form analytical expressions for reflection and transmission coefficients are derived theoretically for appropriate boundary conditions. These expressions are calculated as a non-singular system of linear algebraic equations and depend on the various parameters involved in this non-singular system. Hence,numerical examples are studied to determine the effects of various properties of the sandwich layer on reflection and transmission coefficients. The essential features of layer thickness, incident direction, wave frequency, liquidsaturation and capillary pressure of the porous layer on reflection and transmission coefficients are depicted graphically and discussed. The analysis shows that reflection and transmission coefficients are strongly associated with incident direction and various properties of the porous layer.

关键词： Sandwiched layer Immiscible pore fluids Inhomogeneous wave Reflection and transmission coefficients

current methods for the maturation of induced pluripotent stem cellderived cardiomyocytes

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World journal of Stem cells 2019年第1期11卷 33-43页

作者： Pranav machiraju Steven c Greenway departments of pediatrics and cardiac Sciences Alberta Children’s Hospital Research Institute Libin Cardiovascular Institute of Alberta Cumming School of MedicineUniversity of Calgary departments of pediatrics Cardiac Sciences Biochemistry & Molecular Biology Alberta Children’s Hospital Research Institute Libin Cardiovascular Institute of Alberta Cumming School of Medicine University of Calgary

Induced pluripotent stem cells(iPScs) were first generated by Yamanaka and colleagues over a decade ago. Since then, iPScs have been successfully differentiated into many distinct cell types, enabling tissue-, disease-, and patientspecific in vitro modelling. cardiovascular disease is the greatest cause of mortality worldwide but encompasses rarer disorders of conduction and myocardial function for which a cellular model of study is ideal. Although methods to differentiate iPScs into beating cardiomyocytes(iPSc-cms) have recently been adequately optimized and commercialized, the resulting cells remain largely immature with regards to their structure and function,demonstrating fetal gene expression, disorganized morphology, reliance on predominantly glycolytic metabolism and contractile characteristics that differ from those of adult cardiomyocytes. As such, disease modelling using iPSc-cms may be inaccurate and of limited utility. However, this limitation is widely recognized, and numerous groups have made substantial progress in addressing this problem. This review highlights successful methods that have been developed for the maturation of human iPSc-cms using small molecules,environmental manipulation and 3-dimensional(3 D) growth approaches.

关键词： Induced pluripotent stem cells Induced pluripotent stem cell-derived cardiomyocytes Regenerative medicine Stem cell biology Translational research