Rats were fed 25% casein (25C) diets differing in choline levels (0-0.5%) with and without 0.5% guanidinoacetic acid (GAA) or 0.75% L-methionine for 7 d to determine the effects of dietary choline level on experimental hyperhomocysteinemia. The effects of dietary choline (0.30%) and betaine (0.34%) on GAA- and methionine-induced hyperhomocysteinemia were also compared. Dietary choline suppresse...

Abnormally expanded DNA repeats are associated with several neurodegenerative diseases. In Friedreich's ataxia (FRDA), expanded GAA repeats in intron 1 of the frataxin gene (FXN) reduce FXN mRNA levels in averaged cell samples through a poorly understood mechanism. By visualizing FXN expression and nuclear localization in single cells, we show that GAA-expanded repeats decrease the number of FX...

Apparent shear stress acting on a vertical interface between the main channel and floodplain in a compound channel serves to quantify the momentum transfer between sub sections of this cross section. In this study, three soft computing methods are used to simulate apparent shear stress in prismatic compound channels. The Genetic Algorithm Artificial neural network (GAA), Genetic Programming (GP...

Pompe's disease is caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). GAA is synthesized as a 110-kDa precursor containing N-linked carbohydrates modified with mannose 6-phosphate groups. Following trafficking to the lysosome, presumably via the mannose 6-phosphate receptor, the 110-kDa precursor undergoes a series of complex proteolytic and N-glycan processing events,...

UNLABELLED Pompe's disease (PD) is a metabolic myopathy caused by the accumulation of lysosomal glycogen, secondary to acid alpha-glucosidase (GAA) enzyme deficiency. Childhood and late-onset forms are described, differing by the age of onset and symptoms. In this study were analyzed affected siblings with Pompe's disease (PD) and their distinct clinical and pathological presentations. METHOD...

Recombinant human Acid Alpha Glucosidase (GAA) is the therapeutic enzyme used for the treatment of Pompe disease, a rare genetic disorder characterized by GAA deficiency in the cell lysosomes (Raben et al., Curr Mol Med. 2002; 2:145-166). The manufacturing process for GAA can be challenging, in part due to protease degradation. The overall goal of this study was to understand the effects of GAA...

Expansion of GAA·TTC repeats within the first intron of the frataxin gene is the cause of Friedreich's ataxia (FRDA), an autosomal recessive neurodegenerative disorder. However, no effective treatment for the disease has been developed as yet. In this study, we explored a possibility of shortening expanded GAA repeats associated with FRDA through chemotherapeutically-induced DNA base lesions an...

To identify the relative contribution of local versus regional sources of particulate matter (PM) in the Greater Athens Area (GAA), simultaneous 24-h mass and chemical composition measurements of size segregated particulate matter (PM1, PM2.5 and PM10) were carried out from September 2005 to August 2006 at three locations: one urban (Goudi, Central Athens, “GOU”), one suburban (Lykovrissi, Athe...

Abstract Reliability and controllability for a new scheme of gate-all-around field effect transistor (GAA-FET) with silicon channel utilizing sectorial cross section is evaluated in terms Ion/Ioff current ratio, transconductance, subthreshold slope, threshold voltage roll-off, drain induced barrier lowering (DIBL). In addition, the scaling behavior electronic figures merit comprehensively studi...

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