Babies born with Pompe disease require life-long treatment with enzyme-replacement therapy (ERT). Despite the human origin of the therapy, recombinant human lysosomal acid α glucosidase (GAA, rhGAA), ERT unfortunately leads to the development of high titers of anti-rhGAA antibody, decreased effectiveness of ERT, and a fatal outcome for a significant number of children who have Pompe disease. Th...

In this paper we exploit GAA to build a scheme that converts a simple static password authentication mechanism into a one-time password (OTP) system. The scheme employs a GAA-enabled user device with a display and an input capability (e.g. a 3G mobile phone) and a GAA-aware server. Most importantly, the device does not need to be user or server specific, and can be used in the protocol with no ...

This paper describes the design and optimization of gate-all-around (GAA) MOSFETs structures. The optimum value of Fin width and Fin height are investigated for superior subthreshold behavior. Also the performance of Fin shaped GAA with gate oxide HfO2 are simulated and compared with conventional gate oxide SiO2 for the same structure. As a result, it was observed that the GAA with high K diele...

Friedreich's ataxia (FRDA) is a recessive neurodegenerative disorder commonly associated with hypertrophic cardiomyopathy. FRDA is due to expanded GAA repeats within the first intron of the gene encoding frataxin, a conserved mitochondrial protein involved in iron-sulphur cluster biosynthesis. This mutation leads to partial gene silencing and substantial reduction of the frataxin level. To over...

Expansion of triplex-forming GAA/TTC repeats in the first intron of FXN gene results in Friedreich's ataxia. Besides FXN, there are a number of other polymorphic GAA/TTC loci in the human genome where the size variations thus far have been considered to be a neutral event. Using yeast as a model system, we demonstrate that expanded GAA/TTC repeats represent a threat to eukaryotic genome integri...

The Generic Authentication Architecture (GAA) is a standardised extension to the mobile telephony security infrastructures (including the Universal Mobile Telecommunications System (UMTS) authentication infrastructure) that supports the provision of generic security services to network applications. In this paper we propose one possible means for extending the widespread Trusted Computing secur...

This paper explains the performance analysis of Gate-AllAround silicon nanowire with 80nm diameter field effect transistor based CMOS based device utilizing the 45-nm technology. Simulation and analysis of nanowire (NW) CMOS inverter show that there is the reduction of 70% in leakage power and delay minimization of 25% as compared with 180 nm channel length.Gate-All-Aorund (GAA) configuration p...

OBJECTIVE Friedreich ataxia (FRDA) is an autosomal recessive ataxia resulting from mutations in the frataxin gene (FXN). Such mutations, usually expanded guanine-adenine-adenine (GAA) repeats, give rise to decreased levels of frataxin protein in both affected and unaffected tissues. The goal was to understand the relationship of frataxin levels in peripheral tissues to disease status. METHODS...

OBJECTIVES: The objectives of this project were to determine and quantify the source of bias and error that affect the gross alphaparticle activity (GAA) and the uranium concentration of drinking water samples; determine conditions under which the GAA and the uranium concentration can exceed their true values and cause false-positive violations; provide guidance to water utility, regulatory, an...

Pompe disease (glycogen storage disease type II) is a glycogen storage disease caused by a deficiency of the lysosomal enzyme, acid maltase/acid alpha-1,4 glucosidase (GAA). Deficiency of the enzyme leads primarily to intra-lysosomal glycogen accumulation, primarily in cardiac and skeletal muscles, due to the inability of converting glycogen into glucose. Enzyme replacement therapy (ERT) has be...