Results 11 to 20 of about 526,926 (248)
α-Galactosidase (EC 3.2.1.22) refers to a group of enzymes that hydrolyze oligosaccharides containing α-galactoside-banded glycosides, such as stachyose, raffinose, and verbascose.
Yan Zhao +4 more
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Purification and characterization of an alpha-galactosidase from Aspergillus fumigatus [PDF]
Aspergillus fumigatus secreted invertase (beta-fructofuranosidase) and alpha-galactosidase enzymatic activities able to hydrolyzing raffinose oligosaccharides (RO).
Sebastião Tavares de Rezende +3 more
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Francesco Di Pierro,1 Alexander Bertuccioli,2 Eleonora Marini,3 Leandro Ivaldi4 1Velleja Research, Milan, Italy; 2Italian Association Fitness and Medicine, Fano, PU, Italy; 3Pharmextracta, Pontenure, Piacenza, Italy; 4Digestive Endoscopic Department ...
Di Pierro F +3 more
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Background The pharmacological chaperones therapy is a promising approach to cure genetic diseases. It relies on substrate competitors used at sub-inhibitory concentration which can be administered orally, reach difficult tissues and have low cost ...
Correra Antonella +4 more
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This article is an analysis of the clinical case of differential diagnosis of Fabry disease. Pecular symptoms and the principles of diagnosis of Fabry disease, as well as current treatment options for this disease are presented.
Г. П. Арутюнов +1 more
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Fabry disease is a metabolic alteration linked to an enzymatic deficiency of Alpha-Galactosidase A, this disorder compromises the sphingolipid metabolism, leading to an accumulation of lysosomal globotriaosylceramide and is inherited in an X-linked ...
Jesus Alfredo Uribe-Ardila +1 more
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Evaluation of beta-galactosidase activity in tissue in the presence of blood [PDF]
The reporter gene for beta -galactosidase is frequently used to determine the efficiency of gene transfer in arteries. However, blood is often present in arterial explants and may compromise the results by the presence of hemoglobin. The light absorption
Pelisek, Jaroslav +2 more
core +1 more source
High Throughput Screening for Inhibitors of Alpha-Galactosidase [PDF]
Fabry disease is a rare X-linked lysosomal storage disorder caused by a deficiency in α-galactosidase A (GLA), which catalyzes the hydrolysis of terminal α-galactosyl groups from glycosphingolipids, such as globotriaosylceramide (Gb3). Many of the mutations in the GLA gene are missense alterations that cause misfolding, decreased stability, and/or ...
Motabar, Omid +6 more
openaire +2 more sources
Characterization of small fiber pathology in a mouse model of Fabry disease
Fabry disease (FD) is a life-threatening X-linked lysosomal storage disorder caused by α-galactosidase A (α-GAL) deficiency. Small fiber pathology and pain are major FD symptoms of unknown pathophysiology.
Lukas Hofmann +9 more
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Rapid Immunochromatographic Detection of Serum Anti-α-Galactosidase A Antibodies in Fabry Patients after Enzyme Replacement Therapy. [PDF]
We developed an immunochromatography-based assay for detecting antibodies against recombinant α-galactosidase A proteins in serum. The evaluation of 29 serum samples from Fabry patients, who had received enzyme replacement therapy with agalsidase alpha ...
Sachie Nakano +12 more
doaj +1 more source

