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Properties of mouse alpha-galactosidase.
alpha-Galactosidase has been examined in various murine tissues using the substrate 4-methylumbelliferyl-alpha-galactoside. Mouse liver appears to contain a single major form of the enzyme, as judged by chromatography and electrophoresis. The enzmye was purified 467-fold with a yield of about 40% by a method involving chromatography on Concanavalin A ...
Lusis, A J, Paigen, K
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Corneal alpha-galactosidase deficiency in macular corneal dystrophy
Ophthalmic Paediatrics and Genetics, 1985Glycosidases, which cleave sugar molecules from complex glycopolymers, have been previously quantified in normal human cornea in our laboratory. Data quantifying glycosidases in macular corneal dystrophy are lacking. Tissue obtained at keratoplasty from patients with macular dystrophy and normal corneas obtained from eye bank eyes were used to ...
William E Bruner +2 more
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Alpha‐Galactosidase A Activity Levels in Turkish Male Hemodialysis Patients
Therapeutic Apheresis and Dialysis, 2012AbstractFabry disease is an X‐linked lysosomal storage disorder due to deficient activity of alpha‐galactosidase A (α‐Gal A) leading to renal insufficiency in males. The aim of present study was to investigate the level of α‐Gal A activity and to determine the prevalence of Fabry disease in a Turkish male hemodialysis population. The activity of plasma
Sema Kalkan Uçar +2 more
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Fabry's Disease: Alpha-Galactosidase Deficiency
Science, 1970The leukocytes of male patients with Fabry's disease are deficient in α-galactosidase. The α-galactosidase activity in the leukocytes of female carriers of the disease is 15 to 40 percent of the amount present in normal leukocytes. The activities of β-galactosidase, β-acetylgalactosaminidase, and β-acetylglucosaminidase in the leukocytes of affected ...
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Pseudodeficiency of alpha-galactosidase A.
Clinical genetics, 1982Apparent deficiency of alpha-galactosidase A was observed in a 51-year-old, clinically healthy male, with no clinical symptoms of Fabry disease, and without excess urinary excretion of ceramide trihexoside. The deficiency, which was similar to that found in Fabry disease patients, could be demonstrated using both synthetic and natural substrates.
G, Bach +3 more
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Identification of a novel mutation in the alpha-galactosidase A gene in patients with Fabry disease
OBJECTIVES: Mutation analysis of the alpha-galactosidase A (GLA) gene is a valuable tool for the diagnosis of affected families. In our work, we analyze about one thousand samples per year from patients suspected of having Fabry disease (FD).
Paolo Colomba +2 more
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Immobilized alpha-Galactosidase in the Biochemistry Laboratory
Journal of Chemical Education, 2007This laboratory experiment was designed to demonstrate the application of immobilized galactosidase in food industry to hydrolyze raffinose family oligosaccharides in soymilk. This laboratory experiment was conducted for postgraduate students of biochemistry and developed for graduate and undergraduate students of biochemistry, biotechnology, chemical ...
V. H. Mulimani, K. Dhananjay
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[Microbial alpha-galactosidase (a review)].
Prikladnaia biokhimiia i mikrobiologiia, 1982The review discusses properties, distribution and potential use of microbial alpha-galactosidase (alpha-D-galactoside galactohydrolase, EC 3.2.1.22), the enzyme catalyzing degradation of alpha-D-galactoside bonds. Recent years have witnessed many publications describing microbial alpha-galactosidase which, in contrast to the similar enzyme from higher ...
I V, Ulezlo, O M, Zaprometova
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Novel alpha-galactosidase A mutation in a female with recurrent strokes
Clinical Biochemistry, 2012Anderson-Fabry disease (AFD) is an X-linked inborn error of glycosphingolipid catabolism resulting from the deficient activity of the lysosomal exoglycohydrolase, a-galactosidase A. The complete genomic and cDNA sequences of the human alpha-galactosidase A gene have been determined and to date, several disease-causing alpha-galactosidase A mutations ...
Tuttolomondo A +12 more
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