The most common mutation in Friedreich ataxia is an expanded (GAA*TTC)n sequence, which is highly unstable in human somatic cells and in the germline. The mechanisms responsible for this genetic instability are poorly understood. We previously showed that cloned (GAA*TTC)n sequences replicated in Escherichia coli are more unstable when GAA is the lagging strand template, suggesting erroneous la...

The goal of nanotechnology is to build nanodevices that are intelligent, multifunctional, exceptionally small, extremely sensitive and have low power consumption. When the nanodevice is required for applications such as in vivo biomedical sensors, a nanoscale power source is required. Although a battery or energy storage unit is a choice for powering nanodevices, harvesting energy from the envi...

A 20 band sp3d5s spin-orbit-coupled, semiempirical, atomistic tight-binding model is used with a semiclassical, ballistic field-effect-transistor model, to theoretically examine the bandstructure carrier velocity and ballistic current in silicon nanowire NW transistors. Infinitely long, uniform, cylindrical, and rectangular NWs, of cross sectional diameters/sides ranging from 3–12 nm are consid...

This paper reports quantitative mechanical characterization of silicon carbide (SiC) nanowires (NWs) via in situ tensile tests inside scanning electron microscopy using a microelectromechanical system. The NWs are synthesized using the vapor-liquid-solid process with growth direction of ⟨111⟩. They consist of three types of structures, pure face-centered cubic (3C) structure, 3C structure with ...

We evaluated the effects of exercise on circulating concentrations of guanidinoacetic acid (GAA) and creatine in 23 healthy volunteers subjected to running to exhaustion and free-weight bench-press to volitional failure. Blood was taken before and following each exercise session. Running induced a significant decrease in serum GAA by 20.1% (P < 0.001), while free-weight exercise reduced GAA by ...

Pompe disease results from acid α-glucosidase (GAA) deficiency, and enzyme replacement therapy (ERT) with recombinant human (rh) GAA has clinical benefits, although its limitations include the short half-life of GAA and the formation of antibody responses. The present study compared the efficacy of ERT against gene transfer with an adeno-associated viral (AAV) vector containing a liver-specific...

Guanidinoacetic acid (GAA) conversion to creatine is thought to be involved in cardiometabolic disturbances through its role in biological methylation and insulin secretion. We evaluated the association of serum GAA and creatine with cardiometabolic risk factors in a cohort of 151 apparently healthy adults (82 women and 69 men) aged 18-63 years. Serum levels of GAA and creatine were measured wi...

The thermal conductivity of silicon nanowires (SiNWs) is investigated by molecular dynamics (MD) simulation. It is found that the thermal conductivity of SiNWs can be reduced exponentially by isotopic defects at room temperature. The thermal conductivity reaches the minimum, which is about 27% of that of pure 28Si NW, when doped with 50% isotope atoms. The thermal conductivity of isotopic-super...