Charting the Course for Energy Efficiency in New York: Lessons from Existing Programs

This report examines the performance of the existing suite of energy efficiency efforts run by the New York State Energy Research and Development Authority and the state’s investor owned utilities. The latest data shows that through 2014 EEPS program administrators had achieved 79 percent of their to-date savings goals. The report focuses on the best ways to transition from the EEPS program model to the emerging REV model. Reviewing publicly available information, this analysis takes stock of what the EEPS has achieved and calls for a REV planning and delivery program that builds upon lessons learned from decades of past efforts to achieve self-sustaining efficiency markets. It 1) describes the proposed changes to energy efficiency delivery currently under consideration by the Cuomo Administration, 2) reviews overall EEPS performance through the third quarter of 2014, 3) recommends a framework to serve as the basis for future decision-making, and 4) makes additional recommendations for the future of energy efficiency efforts in New York State

The Fission Fragment Rocket Engine for Mars Fast Transit

In this paper we discuss the advantages and challenges of utilizing Fission Fragment Rocket Engines (FFREs) to dramatically reduce transit time in space travel, for example, traveling to Mars. We discuss methods to decrease the size and weight of FFREs. These include utilizing metallic deuterides as moderators, driving the engines with electron beam bremsstrahlung, and operating the FFREs as subcritical assemblies, not as nuclear reactors. We discuss these and other new innovations based upon improved materials and technology that may be integrated into a revolutionary nuclear rocket technology.Comment: 10 pages, 2 figures, 2 table

Prospects For A New Light-Nuclei, Fission-Fusion Energy Cycle

Future advanced nuclear rocket propulsion, and the availability of new nuclear power cycle designs, will benefit substantially from the large current investment in alternative nuclear energy that is underway today. We propose a new nuclear cycle which includes the primary fission of lithium-6, followed by secondary fusion of deuterium and tritium, and a secondary fission of lithium-7 by tritium. This cycle does not produce nuclear waste from its nuclear fuel, since all byproducts of these cascade reactions are stable, provided that the triton production during the primary reaction is fully consumed in the secondary reactions. This cycle may, however, activate surrounding technical materials from its neutron flux. This light-element nuclear fuel is readily obtained through the ongoing expansion of the lithium mining industry and electric vehicle (EV) battery recycling industries.Comment: 10 pages, 2 figure

Effects of Build Orientation and Heat Treatment on the Porosity Distribution and Morphology within Inconel 625 Fabricated via Laser Powder Bed Fusion

The effects of build orientation, i.e., vertical, or diagonal (45º), and heat treatment on the porosity characteristics within Inconel 625 (IN625) fabricated via laser powder bed fusion (LPBF) was experimentally investigated. Selected samples were heat treated at 1050 ℃ for 1-hour to promote evolution of pores. X-Ray Computed Tomography (XCT) was performed on samples to generate three-dimensional porosity maps. Volume Graphics (VG) software was used to inspect and quantify porosity distributions. Results indicate that build orientation and heat treatment influence measured porosity count. As-built (no heat treatment) sample microstructure was observed to have lower porosity count when compared to heat-treated samples. The vertically built sample was observed to have lower porosity relative to its diagonally built counterpart. The porosity morphology or diameter was observed to vary after heat treatment. On the other hand, the sphericity of pores was not affected by different build orientation and heat treatment.Mechanical Engineerin

Novel pathogenic COX20 variants causing dysarthria, ataxia, and sensory neuropathy.

COX20/FAM36A encodes a mitochondrial complex IV assembly factor important for COX2 activation. Only one homozygous COX20 missense mutation has been previously described in two separate consanguineous families. We report four subjects with features that include childhood hypotonia, areflexia, ataxia, dysarthria, dystonia, and sensory neuropathy. Exome sequencing in all four subjects identified the same novel COX20 variants. One variant affected the splice donor site of intron-one (c.41A>G), while the other variant (c.157+3G>C) affected the splice donor site of intron-two. cDNA and protein analysis indicated that no full-length cDNA or protein was generated. These subjects expand the phenotype associated with COX20 deficiency

Germline IKAROS dimerization haploinsufficiency causes hematologic cytopenias and malignancies

IKAROS is a transcription factor forming homo- and heterodimers and regulating lymphocyte development and function. Germline mutations affecting the IKAROS N-terminal DNA binding domain, acting in a haploinsufficient or dominant-negative manner, cause immunodeficiency. Herein, we describe 4 germline heterozygous IKAROS variants affecting its C-terminal dimerization domain, via haploinsufficiency, in 4 unrelated families. Index patients presented with hematologic disease consisting of cytopenias (thrombocytopenia, anemia, neutropenia)/Evans syndrome and malignancies (T-cell acute lymphoblastic leukemia, Burkitt lymphoma). These dimerization defective mutants disrupt homo- and heterodimerization in a complete or partial manner, but they do not affect the wild-type allele function. Moreover, they alter key mechanisms of IKAROS gene regulation, including sumoylation, protein stability, and the recruitment of the nucleosome remodeling and deacetylase complex; none affected in N-terminal DNA binding defects. These C-terminal dimerization mutations are largely associated with hematologic disorders, display dimerization haploinsufficiency and incomplete clinical penetrance, and differ from previously reported allelic variants in their mechanism of action. Dimerization mutants contribute to the growing spectrum of IKAROS-associated diseases displaying a genotype-phenotype correlation

Isolasi Dan Identifikasi Bakteri Aerob Yang Berpotensi Menjadi Sumber Penularan Infeksi Nosokomial Di Irina a Rsup Prof. Dr. R. D. Kandou Manado

: Nosocomial infection or Hospital Acquired Infection (HAI) is an infection caused by bacteria, parasite, or virus in the hospital, infection occur at least 72 hours since hospitalized. This infection occurs due to lack of hygiene of the environment causing microorganism infection from environment to human, infection can also occur due to transmission of microorganism from one patient to other patients. Inpatients potentially have very high risk of nosocomial infection occur due to continuous requiring treatment for more than 24 hours. Purpose: To determine the existence of aerobic bacteria that could potentially be the source of transmission of nosocomial infection in Irina A RSUP Prof. Dr. R. D. Kandou Manado. Method: This research was descriptive with cross sectional approach. Fourteen samples were taken from the surface of medical equipment, bed, floor, and wall of the treatment room and eight samples were taken from the air. Identification of bacteria was performed by culture on agar medium, staining gram, and biochemical test. Result: Bacillus subtilis found in nine samples (41%), Serratia liquefaciens found in five samples (22,7%), Lactobacillus found in two samples (9,1%), Staphylococcus found in two samples (9,1%), Coccus Gram negative found in two samples (9,1%), Enterobacter aerogenes found in one sample (4,5%), and Enterobacter agglomerans found in one sample (4,5%). Conclusion: Bacillus subtilis is the most bacteria which had been found in this research

Metabolic acetate therapy improves phenotype in the tremor rat model of Canavan disease

Genetic mutations that severely diminish the activity of aspartoacylase (ASPA) result in the fatal brain dysmyelinating disorder, Canavan disease. There is no effective treatment. ASPA produces free acetate from the concentrated brain metabolite, N-acetylaspartate (NAA). Because acetyl coenzyme A is a key building block for lipid synthesis, we postulated that the inability to catabolize NAA leads to a brain acetate deficiency during a critical period of CNS development, impairing myelination and possibly other aspects of brain development. We tested the hypothesis that acetate supplementation during postnatal myelination would ameliorate the severe phenotype associated with ASPA deficiency using the tremor rat model of Canavan disease. Glyceryltriacetate (GTA) was administered orally to tremor rats starting 7 days after birth, and was continued in food and water after weaning. Motor function, myelin lipids, and brain vacuolation were analyzed in GTA-treated and untreated tremor rats. Significant improvements were observed in motor performance and myelin galactocerebroside content in tremor rats treated with GTA. Further, brain vacuolation was modestly reduced, and these reductions were positively correlated with improved motor performance. We also examined the expression of the acetyl coenzyme A synthesizing enzyme acetyl coenzyme A synthase 1 and found upregulation of expression in tremor rats, with a return to near normal expression levels in GTA-treated tremor rats. These results confirm the critical role played by NAA-derived acetate in brain myelination and development, and demonstrate the potential usefulness of acetate therapy for the treatment of Canavan disease

Mutations in the histone methyltransferase gene KMT2B cause complex early-onset dystonia.

Histone lysine methylation, mediated by mixed-lineage leukemia (MLL) proteins, is now known to be critical in the regulation of gene expression, genomic stability, cell cycle and nuclear architecture. Despite MLL proteins being postulated as essential for normal development, little is known about the specific functions of the different MLL lysine methyltransferases. Here we report heterozygous variants in the gene KMT2B (also known as MLL4) in 27 unrelated individuals with a complex progressive childhood-onset dystonia, often associated with a typical facial appearance and characteristic brain magnetic resonance imaging findings. Over time, the majority of affected individuals developed prominent cervical, cranial and laryngeal dystonia. Marked clinical benefit, including the restoration of independent ambulation in some cases, was observed following deep brain stimulation (DBS). These findings highlight a clinically recognizable and potentially treatable form of genetic dystonia, demonstrating the crucial role of KMT2B in the physiological control of voluntary movement.Funding for the project was provided by the Wellcome Trust for UK10K (WT091310) and DDD Study. The DDD study presents independent research commissioned by the Health Innovation Challenge Fund [grant number HICF-1009-003] - see www.ddduk.org/access.html for full acknowledgement. This work was supported in part by the Intramural Research Program of the National Human Genome Research Institute and the Common Fund, NIH Office of the Director. This work was supported in part by the German Ministry of Research and Education (grant nos. 01GS08160 and 01GS08167; German Mental Retardation Network) as part of the National Genome Research Network to A.R. and D.W. and by the Deutsche Forschungsgemeinschaft (AB393/2-2) to A.R. Brain expression data was provided by the UK Human Brain Expression Consortium (UKBEC), which comprises John A. Hardy, Mina Ryten, Michael Weale, Daniah Trabzuni, Adaikalavan Ramasamy, Colin Smith and Robert Walker, affiliated with UCL Institute of Neurology (J.H., M.R., D.T.), King’s College London (M.R., M.W., A.R.) and the University of Edinburgh (C.S., R.W.)