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Developmental regulation of neuronal gene expression by Elongator complex protein 1 dosage

journal of Genetics and Genomics 2022年第7期49卷 654-665页

作者： Elisabetta Morini Dadi Gao Emily M.Logan Monica Salani aram j.krauson Anil Chekuri Yei-Tsung Chen Ashok Ragavendran Probir Chakravarty Serkan Erdin Alexei Stortchevoi jesper Q.Svejstrup Michael E.Talkowski Susan A.Slaugenhaupt Center for Genomic Medicine Massachusetts General Hospital Research InstituteBostonMAUSA Department of Neurology Massachusetts General Hospital Research Institute and Harvard Medical SchoolBostonMAUSA Program in Medical and Population Genetics and Stanley Center for Psychiatric Research Broad Institute of Harvard and MITCambridgeMAUSA Department of Life Sciences and Institute of Genome Sciences National Yang Ming Chiao Tung UniversityTaiwanChina Bioinformatics and Biostatistics The Francis Crick InstituteLondonUK Mechanisms of Transcription Laboratory The Francis Crick InstituteLondonUK Department of Cellular and Molecular Medicine Panum InstituteUniversity of CopenhagenCopenhagenDenmark

Familial dysautonomia(FD), a hereditary sensory and autonomic neuropathy, is caused by a mutation in the Elongator complex protein 1(ELP1) gene that leads to a tissue-specific reduction of ELP1 protein. Our work to generate a phenotypic mouse model for FD headed to the discovery that homozygous deletion of the mouse Elp1 gene leads to embryonic lethality prior to mid-gestation. Given that FD is caused by a reduction, not loss, of ELP1, we generated two new mouse models by introducing different copy numbers of the human FD ELP1 transgene into the Elp1 knockout mouse(Elp1) and observed that human ELP1 expression rescues embryonic development in a dose-dependent manner. We then conducted a comprehensive transcriptome analysis in mouse embryos to identify genes and pathways whose expression correlates with the amount of ELP1. We found that ELP1 is essential for the expression of genes responsible for nervous system development. Further, gene length analysis of the differentially expressed genes showed that the loss of Elp1 mainly impacts the expression of long genes and that by gradually restoring Elongator, their expression is progressively rescued. Finally, through evaluation of co-expression modules, we identified gene sets with unique expression patterns that depended on ELP1 expression.

关键词： ELP1 Elongator Transcriptional elongation Familial dysautonomia Neurodevelopmental disease

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Numerical simulations of smoke spread during solar roof fires

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Building Simulation 2022年第4期15卷 561-570页

作者： Xin Zhang Monireh aram Dahai Qi Liangzhu(Leon)Wang Department of Civil and Building Engineering Universite de Sherbrooke2500boul.de I'UniversiteSherbrooke(Quebec)J1K2R1Canada Centre for Zero Energy Building Studies Deportment of BuildingCivil and Environmental EngineeringConcordia University1455 de Maisonneuve Blvd.WestMontreal(Quebec)H3G 1M8Canada

As the installation of solar roofs increases,so has the concern over *** from a solar roof fire could spread into a building through roof openings and presents a challenge for existing fire protection *** date,there have been insufficient studies on solar roof fire-induced smoke *** this study,we conducted computational fluid dynamics(CDF)simulations using Fire Dynamics Simulator(FDS)to better understand the mechanisms of solar roof fire-induced smoke spread and help with solar roof ***,the photovoltaic(PV)combustion model was created in FDS and validated by experimental data.A parametric study was then simulated to investigate the impacts of roof slopes and vent sizes on the smoke spread of the solar *** was found that the roof slope has a significant effect on the fire smoke *** the roof slope increases,the region of separation,where the smoke and air are mixed,can extend from the leeward side of the building to the roof *** a result,smoke could fill the attic and room more slowly,leading to a lower soot density and lower indoor *** design a solar roof,both fire smoke protection and PV energy performance should be considered,especially for the low latitude regions where the PV optimal title angle regarding energy performance is small and leads to a higher risk of smoke infiltration.

关键词： solar roof fire CFD smoke infiltration

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Incidence, Predictors, Treatment, and Long-Term Prognosis of Patients with Restenosis after Long Drug-Eluting Stent Implantation for Coronary Arteries

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World journal of Cardiovascular Diseases 2014年第13期4卷 631-641页

作者： aram j Mirza Department of Cardiology Sulaimany Cardiac Hospital Sulaimany Region of Kurdistan Iraq

Background: Few data on the clinical course and management of patients experiencing restenosis after implantation of long drug-eluting stents treatment for coronary arteries was available. Objectives: The aim of this study was to evaluate the incidence, predictors, and long-term outcomes of patients with in-stent restenosis (ISR) after percutaneous coronary intervention (PCI) with long (33 mm & 38 mm) drug-eluting stents (DES) for long lesions in coronary arteries including left anterior descending artery (LAD), Lt circumflex artery (Lt Cx), right coronary artery (RCA), obtuse marginal artery (OM) & posterior descending artery (PDA). Methods: Between july 2009 and October 2010, 421 long DES had being implanted in 421 consecutive patients with significant coronary artery stenosis, with 371 patients (88%) undergoing routine follow up, clinical follow up done by exercise stress test at 6 & 12 months after stenting for 126 patients (34%), in 124 patients (33.5%) follow up was done by Computed Tomography angiography & 121 patients (32.5%) with clinically driven angiographic follow-up. A major adverse cardiac event was defined as the composite of death, myocardial infarction (MI), or target-lesion revascularization (TLR) within 15 months. Results: All patients who underwent clinical follow up were asymptomatic. The overall incidence of angiographic (CT or conventional) ISR with long (33 mm & 38 mm) DES was 4% (15 out of 371 stents) with 8 (53.3%) focal-type and 7 (46.7%) with diffuse-type ISR. Six patients (40%) under-went repeated PCI, seven (46.7%) underwent bypass surgery, and 2 (13.3%) were treated medically. During long-term follow-up (ranging from 12 - 26 months), there were no deaths, 3 (0.8%) MI, and 13 (3.5%) repeated target-lesion revascularization (PCI or CABG) cases. The incidence of major adverse cardiac event was 5.3% in the medical group, 10.1% in the repeated PCI group, and 21.4% in the bypass surgery group. Multivariate analysis showed that the occurrence

关键词： Coronary Arteries Restenosis Long Stent

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“Kurdistan” Technique for the Treatment of Unprotected Trifurcation Left Main Stem Coronary Artery Lesion: Case Report

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World journal of Cardiovascular Diseases 2014年第9期4卷 483-491页

作者： aram j. Mirza Department of Cardiology Sulaimany Cardiac Hospital Sulaimany Iraq

The left main coronary artery (LMCA) is responsible for supplying about 75% to 100% of the left ventricular cardiac mass. Left main stem coronary artery (LMCA) disease reduces flow to a large portion of the myocardium, placing the patient at high risk for life-threatening events such as left ventricular dysfunction and arrhythmias with a high mortality approaching 50% in those treated medically. For several decades, coronary artery bypass grafting (CABG) has been considered as a gold standard treatment of unprotected left main coronary artery (ULMCA). However, successful percutaneous coronary interventions (PCI) have been increasingly reported recently due to improved stent technology and better operator expertise. In spite of these factors, management can be challenging especially in LMCA bifurcational & trifurcational lesions, and therefore an integrated approach combining special techniques, physiological evaluation and adjunctive pharmacological agents should be combined for better clinical outcome. Herein, we describe a new promising technique named (Kurdistan technique) for the treatment of trifurcation unprotected left main stem lesion. In the last 18 months, 21 patients with significant trifurcational LMS had underwent PCI using this technique in our hospital (Sulaimany Heart Hospital/Kurdistan). The procedural success rate was 100%, follow up coronary angiography done between 6 - 12 months after the procedure for all the patients with no significant in-stent restenosis in any patient. One case is presented here demonstrating the technique.

关键词： LMS DES Trifurcational

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水痘-带状疱疹病毒毛囊炎促进克隆性淋巴样增生

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世界核心医学期刊文摘（皮肤病学分册） 2006年第1期2卷 23-24页

作者： aram G Rohwedder A Nazeer T j.A. Carlson 党倩丽 Albany Medical College MC-81 47 New Scotland Ave. Albany NY 12208 United States.Dr

Post herpes zoster (HZ) reactions have been associated with panoply of neoplastic, inflammatory, and fibro-inflammatory cutaneous disorders. Varicella zoster virus (VZV) DNA has not been identified in most of these reports. After an episode of HZ, a healthy, active 90-year-old female developed ulcerative nodules in the affected trigeminal V1 dermatome and the contra-lateral trigeminal region over a 1-year period. Excision and/or biopsy of all these lesions showed similar pathologic changes that consisted of herpetic folliculitis, adjacent dense mixed nodular lymphocytic infiltrates with germinal centers (cutaneous lymphoid hyperplasia (CLH)), and in the deeper excision specimens, an obliterative vasculitis of a vessel with smooth muscle in its wall. Immunophenotype analysis revealed a mixed, predominate T-and B-cell population without loss of pan-T cell antigens or aberrant expression by B cells of T-cell antigens. Polymerase chain reaction for herpetic DNA was po sitive for VZV DNA. Lymphocyte gene rearrangement analysis revealed 2 distinct, anatomically and chronologically, monoclonal B-cell populations and a monoclona l T-cell population in one nodule. Treatment with valacyclovir has lead to almo st complete resolution of her cutaneous nodules after 6 months of therapy. In th is case, it can be surmised that persistence of VZV infection and lack of effect ive cell-mediated immunity lead to development of both immunopathology (vasculi tis) and excessive lymphoid cell proliferation (CLH).

关键词：淋巴样克隆性带状疱疹抗原缺失皮肤结节细胞群伐昔洛韦生发中心基因重排细胞过度增生

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Quasi-Chemical Reactions in Irradiated Silicon Crystals with Regard to Ultrafast Irradiation

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journal of Modern Physics 2018年第6期9卷 1271-1280页

作者： Hrant N. Yeritsyan aram A. Sahakyan Norair E. Grigoryan Vachagan V. Harutyunyan Bagrat A. Grigoryan Gayane A. Amatuni Arsham S. Yeremyan Christopher j. Rhodes Department of Applied Physics Yerevan Physics Institute Yerevan Armenia CANDLE Synchrotron Research Institute Yerevan Armenia Fresh-Lands Environmental Actions Caversham UK

This paper reports results from an investigation of the interaction of displaced Si-self atoms (I) and their vacancies (V), with impurities in crystalline silicon (Si), as induced by micro-second pulse duration irradiation with electrons at different energies: 3.5, 14, 25 and 50 MeV and pico-second pulse duration with energy 3.5 MeV. V-V, I-impurity atom and V-impurity atom interactions are analyzed both experimentally and as modeled using computer simulations. A process of divacancy (V2) accumulation in the dose-dependent linear region is investigated. The effect of impurities on recombination of correlated divacancies, and I-atoms that had become displaced from regular lattice points is estimated by computer modeling of an appropriate diffusion-controlled process. It is concluded that the experimental results can be interpreted quantitatively in terms of a strongly anisotropic quasi-one-dimensional diffusion of displaced I-atoms. In addition, a significant difference is found between the effects of pico-second duration electron beam irradiation, which causes the formation of A-centre (V + Oxygen) clusters, while when the beam is applied on a micro-second timescale, divacancies are created instead, although the electrons have the same energy in both cases.

关键词： Silicon Crystal Electron Irradiation Divacancy Radiation Defects Introduction Rate Impurity Atom

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人单核细胞埃里希体病

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世界核心医学期刊文摘（心脏病学分册） 2005年第4期1卷 17-18页

作者： Stone j.H. Dierberg K. aram G. Dumler j. S. 王亭忠 johns Hopkins Vas culitis Center Johns Hopkins Bayview Medical Center 5501 Hopkins Bayview Cir 1B.23 Baltimore MD 21224 United States Dr.

A 56-year-old man with a history of Wegener granulomatosis presented with 6 days of sinus congestion, fever, malaise, myalgias, episcleritis, and a morbilli form rash. An exacerbation of Wegener granulomatosis was the principal concern because of the frequency of flares in that disease. The patient developed acute r enal failure, thrombocytopenia, transaminitis, and, finally, severe myocarditis that led to congestive heart failure. Additional history-taking and the evoluti on of his clinical features led to empirical treatment with doxycycline for huma n monocytic ehrlichiosis(HME). The diagnosis of HME was confirmed by both a poly merase chain reaction assay for Ehrlichia chaffeensis and by the demonstration of morulae within peripheral blood mononuclear cells. The patient improved prompt ly followinginstitution of doxycycline, and his cardiac function returned to nor mal over a period of 4 months.

关键词：人单核细胞埃里希体病韦格纳肉芽肿麻疹样疹巩膜外层炎脱氧土霉素经验性治疗急性心力衰竭外周血单核细胞肌痛

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Total pancreatectomy and islet autotransplantation: A decade nationwide analysis

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World journal of Transplantation 2016年第1期6卷 233-238页

作者： Reza Fazlalizadeh Zhobin Moghadamyeghaneh aram N Demirjian David K Imagawa Clarence E Foster jonathan R Lakey Michael j Stamos Hirohito Ichii Division of Transplant Department of Surgery University of California Irvine School of Medicine Department of Biomedical Engineering University of California

AIM: To investigate outcomes and predictors of inhospital morbidity and mortality after total pancreatectomy(TP) and islet autotransplantation. METHODS: The nationwide inpatient sample(NIS) database was used to identify patients who underwent TP and islet autotransplantation(IAT) between 2002-2012 in the United States. Variables of interest were inherent variables of NIS database which included demographic data(age, sex, and race), comorbidities(such as diabetes mellitus, hypertension, and deficiency anemia), and admission type(elective vs nonelective). The primary endpoints were mortality and postoperative complications according to the ICD-9 diagnosis codes which were reported as the second to 25 th diagnosis of patients in the database. Risk adjusted analysis was performed to investigate morbidity predictors. Multivariate regression analysis was used to identify predictors of in-hospital ***: We evaluated a total of 923 patients who underwent IAT after pancreatectomy during 2002-2012. Among them, there were 754 patients who had TP + IAT. The most common indication ofsurgery was chronic pancreatitis(86%) followed by acute pancreatitis(12%). The number of patients undergoing TP + IAT annually significantly increased during the 11 years of study from 53 cases in 2002 to 155 cases in 2012. Overall mortality and morbidity of patients were 0% and 57.8 %, respectively. Postsurgical hypoinsulinemia was reported in 42.3% of patients, indicating that 57.7% of patients were insulin independent during hospitalization. Predictors of inhospital morbidity were obesity [adjusted odds ratio(AOR): 3.02, P = 0.01], fluid and electrolyte disorders(AOR: 2.71, P < 0.01), alcohol abuse(AOR: 2.63, P < 0.01), and weight loss(AOR: 2.43, P < 0.01). CONCLUSION: TP + IAT is a safe procedure with no mortality, acceptable morbidity, and achieved high rate of early insulin independence. Obesity is the most significant predictor of in-hospital morbidity.

关键词： Total pancreatectomy Pancreatectomy Islet auto transplantation Chronic pancreatitis Insulin independency

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