210 research outputs found

    Quantification of execution and emission efficiency of a fueled diesel engine

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    A significant portion of the automobile and industrial sector is mostly dependent on the running of diesel engines as it is efficient and shifts a large of the goods around the globe and power various equipment. Also, as the use of energy as diesel fuel is increasing enormously with the expansion of industrial growth, diversification, this led to the accelerating global emissions, global climatic change, health issues, and exhaustion of fuels. To succumb this, alternative fuel is needed to fight against the ill effects and as a replacement to diesel fuel. Thus, vegetable oils as alternative fuels are drawing more attention as they are renewable and do not address the problem of greenhouse gas. In the present work, the cottonseed oil was chosen as the favorite among the vegetable oils due to its advantages like less pollutant level, excessive availability, etc. The transesterification process was used to produce the cottonseed oil biodiesel. This research aims to investigate efficiency, emission characteristics by using smooth diesel, cottonseed oil, and mixtures with varying composition from 20 % to 80 % in 20 % steps to identify sustainable fuel as a substitute for existing fuel and to overcome fuel demand and enviro effects. This test was conducted on single-cylinder four-stroke water-cooled diesel engines. From the results, it was revealed that cottonseed oil and its blends have a significant influence on performance and emission characters

    Insilico analysis of Arabidopsis ferric reductase oxidases (FRO) proteins associated with iron homeostasis

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    The ferric reduction oxidase (FRO) gene family is involved in various biological processes of plants and plays an essential role in metal homeostasis, tolerance, and signaling networks in response to several abiotic stresses. Our study describes the structural, functional characterization, and evolutionary relationships of eight Arabidopsis FRO proteins. The studies predicted the subcellular localization of FRO proteins to the plasma membrane, mitochondria, and chloroplast organelles. The structural analysis revealed localization of proteins onto the first and fifth chromosomes having 8-9exons and 8-10 transmembrane helices. The protein features of FRO proteins revealed 699-747 amino acids having 79600.02-84126.3 (Da) molecular weight. The six highly conserved protein motifs were predicted with 45-50 amino acids long representing ferric chelate reductase family domains. The phylogeny tree constructed using Clustal W divided the FRO proteins into two clusters and the interactome network revealed the co-expression of COPT1, NRAMP1, NRAMP3, NRAMP4, FRD3, OPT3, IRT1, IRT2, ZIF1, PYE proteins along with the seven FRO proteins

    Heat content of the Arabian Sea Mini Warm Pool is increasing

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    © The Author(s), 2015. This article is distributed under the terms of the Creative Commons Attribution License. The definitive version was published in Atmospheric Science Letters 17 (2016): 39-42, doi:10.1002/asl.596.Sea surface temperature in the Arabian Sea Mini Warm Pool has been suggested to be one of the factors that affects the Indian summer monsoon. In this paper, we analyze the annual ocean heat content (OHC) of this region during 1993–2010, using in situ data, satellite observations, and a model simulation. We find that OHC increases significantly in the region during this period relative to the north Indian Ocean, and propose that this increase could have caused the decrease in Indian Summer Monsoon Rainfall that occurred at the same time

    Increased RPA1 gene dosage affects genomic stability potentially contributing to 17p13.3 duplication syndrome

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    A novel microduplication syndrome involving various-sized contiguous duplications in 17p13.3 has recently been described, suggesting that increased copy number of genes in 17p13.3, particularly PAFAH1B1, is associated with clinical features including facial dysmorphism, developmental delay, and autism spectrum disorder. We have previously shown that patient-derived cell lines from individuals with haploinsufficiency of RPA1, a gene within 17p13.3, exhibit an impaired ATR-dependent DNA damage response (DDR). Here, we show that cell lines from patients with duplications specifically incorporating RPA1 exhibit a different although characteristic spectrum of DDR defects including abnormal S phase distribution, attenuated DNA double strand break (DSB)-induced RAD51 chromatin retention, elevated genomic instability, and increased sensitivity to DNA damaging agents. Using controlled conditional over-expression of RPA1 in a human model cell system, we also see attenuated DSB-induced RAD51 chromatin retention. Furthermore, we find that transient over-expression of RPA1 can impact on homologous recombination (HR) pathways following DSB formation, favouring engagement in aberrant forms of recombination and repair. Our data identifies unanticipated defects in the DDR associated with duplications in 17p13.3 in humans involving modest RPA1 over-expression

    Growth characteristics in individuals with osteogenesis imperfecta in North America: results from a multicenter study.

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    PurposeOsteogenesis imperfecta (OI) predisposes people to recurrent fractures, bone deformities, and short stature. There is a lack of large-scale systematic studies that have investigated growth parameters in OI.MethodsUsing data from the Linked Clinical Research Centers, we compared height, growth velocity, weight, and body mass index (BMI) in 552 individuals with OI. Height, weight, and BMI were plotted on Centers for Disease Control and Prevention normative curves.ResultsIn children, the median z-scores for height in OI types I, III, and IV were -0.66, -6.91, and -2.79, respectively. Growth velocity was diminished in OI types III and IV. The median z-score for weight in children with OI type III was -4.55. The median z-scores for BMI in children with OI types I, III, and IV were 0.10, 0.91, and 0.67, respectively. Generalized linear model analyses demonstrated that the height z-score was positively correlated with the severity of the OI subtype (P < 0.001), age, bisphosphonate use, and rodding (P < 0.05).ConclusionFrom the largest cohort of individuals with OI, we provide median values for height, weight, and BMI z-scores that can aid the evaluation of overall growth in the clinic setting. This study is an important first step in the generation of OI-specific growth curves

    ASL expression in ALDH1A1+ neurons in the substantia nigra metabolically contributes to neurodegenerative phenotype

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    Argininosuccinate lyase (ASL) is essential for the NO-dependent regulation of tyrosine hydroxylase (TH) and thus for catecholamine production. Using a conditional mouse model with loss of ASL in catecholamine neurons, we demonstrate that ASL is expressed in dopaminergic neurons in the substantia nigra pars compacta, including the ALDH1A1 + subpopulation that is pivotal for the pathogenesis of Parkinson disease (PD). Neuronal loss of ASL results in catecholamine deficiency, in accumulation and formation of tyrosine aggregates, in elevation of α-synuclein, and phenotypically in motor and cognitive deficits. NO supplementation rescues the formation of aggregates as well as the motor deficiencies. Our data point to a potential metabolic link between accumulations of tyrosine and seeding of pathological aggregates in neurons as initiators for the pathological processes involved in neurodegeneration. Hence, interventions in tyrosine metabolism via regulation of NO levels may be therapeutic beneficial for the treatment of catecholamine-related neurodegenerative disorders

    Transatlantic combined and comparative data analysis of 1095 patients with urea cycle disorders?A successful strategy for clinical research of rare diseases

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    BACKGROUND: To improve our understanding of urea cycle disorders (UCDs) prospectively followed by two North American (NA) and European (EU) patient cohorts. AIMS: Description of the NA and EU patient samples and investigation of the prospects of combined and comparative analyses for individuals with UCDs. METHODS: Retrieval and comparison of the data from 1095 individuals (NA: 620, EU: 475) from two electronic databases. RESULTS: The proportion of females with ornithine transcarbamylase deficiency (fOTC-D), particularly those being asymptomatic (asfOTC-D), was higher in the NA than in the EU sample. Exclusion of asfOTC-D resulted in similar distributions in both samples. The mean age at first symptoms was higher in NA than in EU patients with late onset (LO), but similar for those with early (</= 28 days) onset (EO) of symptoms. Also, the mean age at diagnosis and diagnostic delay for EO and LO patients were similar in the NA and EU cohorts. In most patients (including fOTC-D), diagnosis was made after the onset of symptoms (59.9%) or by high-risk family screening (24.7%), and less often by newborn screening (8.9%) and prenatal testing (3.7%). Analysis of clinical phenotypes revealed that EO patients presented with more symptoms than LO individuals, but that numbers of symptoms correlated with plasma ammonium concentrations in EO patients only. Liver transplantation was reported for 90 NA and 25 EU patients. CONCLUSIONS: Combined analysis of databases drawn from distinct populations opens the possibility to increase sample sizes for natural history questions, while comparative analysis utilizing differences in approach to treatment can evaluate therapeutic options and enhance long-term outcome studies

    BAFopathies\u27 DNA methylation epi-signatures demonstrate diagnostic utility and functional continuum of Coffin-Siris and Nicolaides-Baraitser syndromes.

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    Coffin-Siris and Nicolaides-Baraitser syndromes (CSS and NCBRS) are Mendelian disorders caused by mutations in subunits of the BAF chromatin remodeling complex. We report overlapping peripheral blood DNA methylation epi-signatures in individuals with various subtypes of CSS (ARID1B, SMARCB1, and SMARCA4) and NCBRS (SMARCA2). We demonstrate that the degree of similarity in the epi-signatures of some CSS subtypes and NCBRS can be greater than that within CSS, indicating a link in the functional basis of the two syndromes. We show that chromosome 6q25 microdeletion syndrome, harboring ARID1B deletions, exhibits a similar CSS/NCBRS methylation profile. Specificity of this epi-signature was confirmed across a wide range of neurodevelopmental conditions including other chromatin remodeling and epigenetic machinery disorders. We demonstrate that a machine-learning model trained on this DNA methylation profile can resolve ambiguous clinical cases, reclassify those with variants of unknown significance, and identify previously undiagnosed subjects through targeted population screening
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