It is reported that gossypol acetate (GAA) has obvious effects on inhibiting the growth of tumors, by inhibiting the activity of enzymes. Ultrastructural study showed that GAA can cause morphological changes of mitochondria which leads to the apoptosis of tumors. However, little is known about the pathways that how the GAA triggers apoptosis of tumors and what kind of the molecular events have ...

Glycogen storage disease type II (GSDII) is a lysosomal storage disease caused by a deficiency in acid alpha-glucosidase (GAA), and leads to cardiorespiratory failure by the age of 2 years. In this study, we investigate the impact of anti-GAA antibody formation on cross-correction of the heart, diaphragm, and hind-limb muscles from liver-directed delivery of recombinant adeno-associated virus (...

Pompe disease is an autosomal recessive disorder caused by a deficiency of acid α-glucosidase (GAA), an enzyme responsible for hydrolyzing lysosomal glycogen. Deficiency of GAA leads to systemic glycogen accumulation in the lysosomes of skeletal muscle, motor neurons, and smooth muscle. Skeletal muscle and motor neuron pathology are known to contribute to respiratory insufficiency in Pompe dise...

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...

In quest of new (”post-CMOS”) switches, in particular for ultra-low power application, the Tunnel Field Effect Transistor (TFET) [1] raised a lot of attention. The working principle of this device is the generation of electron-hole pairs by band-to-band tunneling (BTBT) between the valence band (VB) and the conduction band (CB). Thus, contrary to the common MOSFET, the source of current in a TF...

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...