The present study aims to determine the best technique of Guanidinoacetic acid (GAA) supplementation improve retention added GAA after immersion in water on growth performance Pangasius catfish (Pangasius hypophthalmus). juveniles with a mean body weight and length 16.05 ± 0.47 g 12.42 0.30 cm were randomly distributed into 9 pond units (2 x 1 0,6 m). Three experimental diets formulated contain...

Pompe disease, also known as glycogen storage disease (GSD) type II, is caused by deficiency of lysosomal acid α-glucosidase (GAA). The resulting glycogen accumulation causes a spectrum of disease severity ranging from a rapidly progressive course that is typically fatal by 1 to 2 years of age to a slower progressive course that causes significant morbidity and early mortality in children and a...

Friedreich ataxia (FRDA) is caused by hyperexpansion of GAA*TTC repeats located in the first intron of the FXN gene, which inhibits transcription leading to the deficiency of frataxin. The FXN gene is an excellent target for therapeutic intervention since (i) 98% of patients carry the same type of mutation, (ii) the mutation is intronic, thus leaving the FXN coding sequence unaffected and (iii)...

Pompe disease is a fatal genetic muscle disorder caused by a deficiency of acid a -glucosidase (GAA), a glycogen degrading lysosomal enzyme. GAA-deficient (AMD) Japanese quails exhibit progressive myopathy and cannot lift their wings, fly, or right themselves from the supine position (flip test). Six 4-wk-old acid maltase–deficient quails, with the clinical symptoms listed, were intravenously i...

BACKGROUND Mutations in the acid alpha-glucosidase (GAA) gene usually lead to reduced GAA activity. In this study, we analyzed the mutations of GAA and GAA enzyme activity from one sibling suspected Pompe disease and their first-degree relatives. MATERIALS AND METHODS In this cross-sectional study, GAA enzyme activity assay was assessed using tandem mass spectrometry. Polymerase chain reactio...

This study evaluates the effects of different levels of guanidinoacetic acid (GAA) supplement on growth performance and gut morphology in broilers (Ross 308 strain) raised at high altitude (2100 m). A total of 300 one-day-old male broiler chicks (Ross 308 strain) were used in a completely randomized design with five treatments and four replicate pens of 15 birds in each. Five dietary treatments...

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...

Pompe disease is a fatal genetic muscle disorder caused by a deficiency of acid alpha-glucosidase (GAA), a glycogen degrading lysosomal enzyme. GAA-deficient (AMD) Japanese quails exhibit progressive myopathy and cannot lift their wings, fly, or right themselves from the supine position (flip test). Six 4-wk-old acid maltase-deficient quails, with the clinical symptoms listed, were intravenousl...

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...

Methionine is the main source of methyl groups that are partitioned to synthesize various methylated products including creatine, phosphatidylcholine (PC), and methylated DNA. Whether increased methylation of 1 product can divert methionine from protein synthesis or other methylation products was the aim of this experiment. We used an excess of guanidinoacetate (GAA) to synthesize creatine to c...