Effects of lysosomal biotherapeutic recombinant protein expression on cell stress and protease and general host cell protein release in Chinese hamster ovary cells
Sign Up to like & getrecommendations! Published in 2017 at "Biotechnology Progress"
DOI: 10.1002/btpr.2455
Abstract: 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.… read more here.
Keywords: protein; protease; gaa; cell ... See more keywords
Antibody-mediated enzyme replacement therapy targeting both lysosomal and cytoplasmic glycogen in Pompe disease
Sign Up to like & getrecommendations! Published in 2017 at "Journal of Molecular Medicine"
DOI: 10.1007/s00109-017-1505-9
Abstract: Pompe disease is characterized by accumulation of both lysosomal and cytoplasmic glycogen primarily in skeletal and cardiac muscles. Mannose-6-phosphate receptor-mediated enzyme replacement therapy (ERT) with recombinant human acid α-glucosidase (rhGAA) targets the enzyme to lysosomes… read more here.
Keywords: antibody; glycogen; pompe disease; gaa ... See more keywords
Continued analysis of GAA mice treated with novel hybrid promoter rAAV vectors expressing acid alpha-glucosidase
Sign Up to like & getrecommendations! Published in 2019 at "Molecular Genetics and Metabolism"
DOI: 10.1016/j.ymgme.2018.12.168
Abstract: Pompe disease is caused by mutations in the acid alpha-glucosidase gene (GAA) that is responsible for processing lysosomal glycogen. Patients with Pompe disease exhibit clinical phenotypes across a variety of tissues, including glycogen buildup in… read more here.
Keywords: mice; muscle; gaa; raav vectors ... See more keywords
Identification of Gossypol Acetate as an Autophagy Modulator with Potent Anti-tumor Effect against Cancer Cells.
Sign Up to like & getrecommendations! Published in 2022 at "Journal of agricultural and food chemistry"
DOI: 10.1021/acs.jafc.1c06399
Abstract: Autophagy, an evolutionarily conserved process, is intricately involved in many aspects of human health and a variety of human diseases, including cancer. Discovery of small-molecule autophagy modulators with potent anticancer effect would be of great… read more here.
Keywords: cancer cells; effect; gaa; gossypol acetate ... See more keywords
Performance, antioxidant status, nutrient retention and serum profile responses of laying Japanese quails to increasing addition levels of dietary guanidinoacetic acid
Sign Up to like & getrecommendations! Published in 2019 at "Italian Journal of Animal Science"
DOI: 10.1080/1828051x.2019.1698325
Abstract: Abstract In this study, the effects of different levels of dietary guanidinoacetic acid (GAA) supplementation (0, 0.6, 1.2 and 1.8 g/kg) in laying quails was evaluated in a completely randomised design comprised of four dietary treatments… read more here.
Keywords: observed gaa; performance; supplementation; antioxidant status ... See more keywords
Effects of dietary supplementation of guanidinoacetic acid on physiological response of broiler chicken exposed to repeated lactic acid injection
Sign Up to like & getrecommendations! Published in 2021 at "Italian Journal of Animal Science"
DOI: 10.1080/1828051x.2021.1873075
Abstract: Abstract Increased blood lactic acid is likely to be involved in the incidence of sudden death syndrome (SDS) in broiler chicken. Guanidinoacetic acid (GAA) with direct or indirect influence on the cardiovascular system may provide,… read more here.
Keywords: guanidinoacetic acid; broiler; broiler chicken; gaa ... See more keywords
Disruption of the gaa Gene in Zebrafish Fails to Generate the Phenotype of Classical Pompe Disease.
Sign Up to like & getrecommendations! Published in 2017 at "DNA and cell biology"
DOI: 10.1089/dna.2016.3459
Abstract: The underlying pathogenic lesions of glycogen storage disease type II (GSD II) and the diversity of this disease among different species are still under exploration. Thus, we created an acid alpha-glucosidase (gaa) gene-mutated zebrafish model… read more here.
Keywords: disease; disruption gaa; gene zebrafish; gaa ... See more keywords
Systemic Delivery of AAVB1-GAA Clears Glycogen and Prolongs Survival in a Mouse Model of Pompe Disease.
Sign Up to like & getrecommendations! Published in 2019 at "Human gene therapy"
DOI: 10.1089/hum.2018.016
Abstract: Pompe disease is an autosomal recessive glycogen storage disorder caused by deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). GAA deficiency results in systemic lysosomal glycogen accumulation and cellular disruption in muscle and the central… read more here.
Keywords: aavb1 gaa; gaa; glycogen; pompe disease ... See more keywords
Relative bioavailability of guanidinoacetic acid delivered ruminally or abomasally to cattle.
Sign Up to like & getrecommendations! Published in 2020 at "Journal of animal science"
DOI: 10.1093/jas/skaa282
Abstract: This study assessed relative bioavailability of guanidinoacetic acid (GAA) in cattle. Seven ruminally cannulated Holstein steers (initial body weight of 280 kg) were used in an experiment with a 5 × 5 Latin square design; the… read more here.
Keywords: relative bioavailability; guanidinoacetic acid; infusion; bioavailability guanidinoacetic ... See more keywords
Effects of guanidinoacetic acid supplementation on nitrogen retention and methionine flux in cattle.
Sign Up to like & getrecommendations! Published in 2021 at "Journal of animal science"
DOI: 10.1093/jas/skab172
Abstract: Creatine stores high-energy phosphate bonds in muscle and is synthesized in the liver through methylation of guanidinoacetic acid (GAA). Supplementation of GAA may therefore increase methyl group requirements, and this may affect methyl group utilization.… read more here.
Keywords: supplementation; guanidinoacetic acid; gaa supplementation; supplementation gaa ... See more keywords
Effects of guanidinoacetic acid on growth performance, creatine and energy metabolism, and carcass characteristics in growing-finishing pigs.
Sign Up to like & getrecommendations! Published in 2018 at "Journal of animal science"
DOI: 10.1093/jas/sky186
Abstract: The aim of this study was to investigate the effects of dietary supplementation with guanidinoacetic acid (GAA) on the growth performance, creatine and energy metabolism, and carcass characteristics in growing-finishing pigs. In Exp. 1, Duroc… read more here.
Keywords: gaa; dietary gaa; carcass; growth performance ... See more keywords