Pompe disease is an autosomal recessive lysosomal storage disorder caused by deficiency of acid alpha-glucosidase (GAA) activity, leading to the progressive accumulation glycogen in lysosomes skeletal and cardiac muscles. An pseudodeficiency allele a change GAA gene sequence that results enzyme activity reduction, but does not cause disease. In Japan Taiwan, there high prevalence (c.1725G > A c...

In this study, we evaluated how guanidinoacetic acid (GAA) addition in diets with various metabolizable energy (ME) contents affects the performance of broiler chickens. We also estimated equivalence GAA ME. distributed 1,280 one-day-old broilers a completely randomized design eight treatments, replicates, and twenty birds per experimental unit. Treatments were based on ME levels (2,775-2,875-2...

Mycoplasma gallisepticum, the cause of chronic respiratory infections in the avian host, possesses a family of M9/pMGA genes encoding an adhesin(s) associated with hemagglutination. Nucleotide sequences of M9/pMGA gene family members indicate extensive sequence similarity in the promoter regions of both the transcribed and silent genes. The mechanism that regulates M9/pMGA gene expression is un...

In addition to its use in plain future assertions, gaa is also used to make epistemic modal claims that lack future orientation. (2a) is an epistemic modal claim with present temporal orientation. The indicative non-modal counterpart of (2a) is (2b). In (2a) the auxiliary bears subjunctive mood morphology and the particle gaa, whereas in (2b) the present indicative auxiliary hai is used. The fe...

Interfacial As is shown to reduce reverse-bias current in AI-GaAs Schottky barriers. It is suggested that the leakage reduction is associated with the removal oflow work function phases at the interface. In addition, current-voltage measurements performed on Ino.75 Gaa.25 As-GaAs heterojunctions indicate a dependence upon the condition of the GaAs prior to deposition of the Ino.75 Gaa.25 As layer.

A polymeric nanogel has been used to sequester and turn off a lysosomal protein, acid α-glucosidase (GAA). The nanogel contains a β-thiopropionate cross-linker, which endows the nanogel with pH-sensitivity. While encapsulation of the enzyme fully turns off its activity, approximately 75% of the activity is recovered upon reducing the pH to 5.0. The recovered activity is ascribed to pH-induced d...

We have recently shown that GAA repeats severely impede replication elongation during the first replication cycle of transfected DNA wherein the chromatin is still at the formation stage.1 Here we extend this study by showing that two GAA repeats located within the same plasmid in the direct orientation can form complexes upon transient transfection of mammalian Cos-1 cells. However, these comp...

Frataxin, a highly conserved protein found in prokaryotes and eukaryotes, is required for efficient regulation of cellular iron homeostasis. Humans with a frataxin deficiency have the cardio- and neurodegenerative disorder Friedreich's ataxia, commonly resulting from a GAA trinucleotide repeat expansion in the frataxin gene. While frataxin's specific function remains a point of controversy, the...

Gambieric acid A (GAA) and its congeners belong to the family of marine polycyclic ether natural products. Their highly complex molecular architecture and unique biological activities have been of intense interest within the synthetic community. We have previously reported the first total synthesis, stereochemical reassignment, and preliminary structure-activity relationships of GAA. Here we di...

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