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Mucopolysaccharidosis type IIIA (MPS-IIIA) or Sanfilippo A syndrome is a lysosomal storage genetic disease that results from the deficiency of the N-sulfoglucosamine sulfohydrolase (SGSH) protein, a sulfamidase required for the degradation of heparan sulfate glycosaminoglycans (GAGs). The accumulation of these macromolecules leads to somatic organ pathologies, severe neurodegeneration and death. To assess a novel gene therapy approach based on prolonged secretion of the missing enzyme by the liver, mediated by hydrodynamic gene delivery, we first compared a kanamycin and an antibiotic-free expression plasmid vector, called pFAR4. Thanks to the reduced vector size, pFAR4 derivatives containing either a ubiquitous or a liver-specific promoter mediated a higher reporter gene expression level than the control plasmid. Hydrodynamic delivery of SGSH-encoding pFAR4 into MPS-IIIA diseased mice led to high serum levels of sulfamidase protein that was efficiently taken up by neighboring organs, as shown by the correction of GAG accumulation. A similar reduction in GAG content was also observed in the brain, at early stages of the disease. Thus, this study contributes to the effort towards the development of novel biosafe non-viral gene vectors for therapeutic protein expression in the liver, and represents a first step towards an alternative gene therapy approach for the MPS-IIIA disease.Gene Therapy advance online publication, 21 August 2014; doi:10.1038/gt.2014.75. Source


Rismanchi B.,University of Malaya | Saidur R.,University of Malaya | Saidur R.,CNRS Health Technology, Chemistry and Biology Unit | Boroumandjazi G.,University of Malaya | Ahmed S.,University of Malaya
Renewable and Sustainable Energy Reviews | Year: 2012

As the air conditioning system is one of the largest contributors to electrical peak demand, the role of the cold thermal energy storage (CTES) system has become more significant in the past decade. The present paper has reviewed the studies conducted on the energy and exergy analysis of CTES systems with a special focus on ice thermal and chilled water storage systems as the most common types of CTES. However, choosing a proper CTES technique is mainly dependent on localized parameters such as the ambient temperature profile, electricity rate structure, and user's habit, which makes it quite difficult and complicated as it depends on many individual parameters. Therefore, it was found that energy and exergy analysis can help significantly for a better judgment. The review paper has shown that the exergetic efficiency analysis can show a more realistic picture than energy efficiency analysis. In addition, the environmental impact and the economic feasibility of these systems are also investigated. It was found that, based on the total exergy efficiency, the ice on coil (internal melt) is known as the most desirable CTES system. © 2012 Elsevier Ltd. Source


Aman M.M.,University of Malaya | Jasmon G.B.,University of Malaya | Bakar A.H.A.,University of Malaya | Mokhlis H.,University of Malaya | Mokhlis H.,CNRS Health Technology, Chemistry and Biology Unit
International Journal of Electrical Power and Energy Systems | Year: 2014

This paper presents a new algorithm for network reconfiguration based on maximization of system loadability. Bifurcation theorem known as Continuation Power Flow (CPF) theorem and radial distribution load flow analysis are used to find the maximum loadability point. Network reconfiguration results are also compared with existing technique proposed in literature. In the proposed method, to find the optimum tie-switch position, a Discrete Artificial Bee Colony (DABC) approach is applied. Graph theory is used to ensure the radiality of the system. The proposed algorithm is tested on 33-bus and 69-bus radial distribution networks, each having 5-tie switches. The result shows that using the proposed method the kVA margin to maximum loading (KMML) increases, overall voltage profile also improved and the distribution system can handle more connected load (kVA) without violating the voltage and line current constraints. Results further show that the voltage limit is an important factor than the line current constraints in adding further load to the buses. © 2013 Elsevier Ltd. All rights reserved. Source


Anisur M.R.,University of Malaya | Mahfuz M.H.,University of Malaya | Kibria M.A.,University of Malaya | Saidur R.,University of Malaya | And 3 more authors.
Renewable and Sustainable Energy Reviews | Year: 2013

The application of thermal energy storage (TES) system with phase change material (PCM) is an effective way for energy conservation and greenhouse gas (GHG) emission reduction. Global warming is increasing along with the energy consumption. Many researchers are concerned about this present global environmental problem for fossil-fuel burning. Thermal energy storage system with phase change material is observed as a potential candidate for mitigating this problem. This paper emphasizes the opportunities for energy savings and greenhouse-gas emissions reduction with the implementation of PCM in TES systems. For instance, about 3.43% of CO2 emission by 2020 could be reduced through the application of PCM in building and solar thermal power systems. Similarly, energy conservation and GHGs emission reduction by other PCM applications for thermal comfort of vehicles, transport refrigeration, engine cold start, greenhouse and waste heat management are also presented. In addition, some present investigations on the performance improvement of the phase change materials are addressed. © 2012 Elsevier Ltd. All rights reserved. Source


Raymond W.J.K.,University of Malaya | Illias H.A.,University of Malaya | Bakar A.H.A.,CNRS Health Technology, Chemistry and Biology Unit | Mokhlis H.,University of Malaya
Measurement: Journal of the International Measurement Confederation | Year: 2015

It is well known that a correlation exist between the pattern of partial discharge (PD) behavior and the insulation quality. Since different sources of partial discharge have their own unique effects on the degradation of insulation material, it is vital to investigate the relationship between the defect type and the PD to determine the insulation quality. Numerous work had been done to classify partial discharge patterns with variable success. Past research work in partial discharge classification varies greatly in terms of classification techniques used, choice of feature extraction, denoising method, training process, artificial defects created for training purposes and performance assessment. Therefore it is necessary for a literature survey to access the state of the art development in partial discharge classification. © 2015 Elsevier Ltd.All rights reserved. Source

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