Glycogen storage disease type II (Pompe disease) causes death in infancy from cardiorespiratory failure due to acid alpha-glucosidase (GAA; acid maltase) deficiency. An AAV2 vector pseudotyped as AAV6 (AAV2/6 vector) transiently expressed high-level human GAA in GAA-knockout (GAA-KO) mice without reducing glycogen storage; however, in immunodeficient GAA-KO/SCID mice the AAV2/6 vector expressed...

We introduce the Generic Agent Architecture (GAA) along with Biter—an implementation of our GAA for the RoboCup domain. The GAA incorporates an elegant object-oriented design meant to handle the type of activities typical for an agent in a multiagent system. These activities include reactive responses, long-term behaviors, and conversations with other agents. We also show how small modification...

The tunneling field-effect transistor (TFET) is one of the most promising candidates for future low-power electronics because of its potential to achieve a subthreshold swing less than the 60 mV/decade thermal limit at room temperature. It can surpass this limit because the turn-on of tunneling does not sample the Maxwell-Boltzmann distribution of carriers that gives rise to the 60 mV/decade li...

Though silicon tunnel field effect transistor (TFET) has attracted attention for sub-60mV/decade subthreshold swing and very small OFF current (IOFF), its practical application is questionable due to low ON current (ION) and complicated fabrication process steps. In this paper, a new n-type classical-MOSFET-alike tunnel FET architecture is proposed, which offers sub-60mV/decade subthreshold swi...

The progress in the synthesis of nanomaterials allows the realization of low-dimensional thermoelectric devices based on 1D nanowires (NWs) and 2D superlattices. These confined systems offer the possibility of partially engineering the electronic and phononic dispersions and scattering mechanisms. Thus, the electrical and thermal conductivity, and the Seebeck coefficient can be designed to some...

OBJECTIVES Guanidinoacetic acid (GAA) is a natural precursor of creatine, yet the potential use of GAA as a nutritional additive for restoring creatine availability in humans has been limited by unclear efficacy and safety after exogenous GAA administration. The present study evaluated the effects of orally administered GAA on serum and urinary GAA, creatine and creatinine concentration, and on...

We have used a peptide-based targeting system to improve lysosomal delivery of acid α-glucosidase (GAA), the enzyme deficient in patients with Pompe disease. Human GAA was fused to the glycosylation-independent lysosomal targeting (GILT) tag, which contains a portion of insulin-like growth factor II, to create an active, chimeric enzyme with high affinity for the cation-independent mannose 6-ph...

Semiconducting nanowires (NWs) are becoming essential nanobuilding blocks for advanced devices from sensors to energy harvesters, however their full technology penetration requires large scale materials synthesis together with efficient NW assembly methods. We demonstrate a scalable one-step solution process for the direct selection, collection, and ordered assembly of silicon NWs with desired ...