Newborn Screening for Lysosomal Storage Disorders in Belgium
Lysosomal storage disorders (LSDs) are a group of metabolic disorders with various clinical presentations, which complicate diagnosis. A pilot study was performed to test the appropriateness and effectiveness of the newborn screening method for Pompe ...
Francois Eyskens MD, PhD +1 more
doaj +1 more source
A Novel Alkaline α-Galactosidase from Melon Fruit with a Substrate Preference for Raffinose1
The cucurbits translocate the galactosyl-sucrose oligosaccharides raffinose and stachyose, therefore, α-galactosidase (α-d-galactoside galactohydrolase, EC 3.2.1.22) is expected to function as the initial enzyme of photoassimilate catabolism.
Zhifang Gao, Arthur A. Schaffer
core +1 more source
α-Galactosidase production by a novel actinomycete strain Streptomyces griseoloalbus in shake flask culture was optimized using response surface methodology.
Grace Sathyanesan Anisha +2 more
doaj
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
Purification and some properties of phospho-β-galactosidase from the Gram-negative oral bacterium Leptotrichia buccalis ATCC 14201 [PDF]
Phospho-beta-galactosidase (P-beta-gal; EC 3.2.1.85) is induced during growth of Leptotrichia buccalis ATCC 14201 on lactose and lactulose. The enzyme has been purified to electrophoretic homogeneity (M(r) approximately 53 kDa, pI approximately 4.8), and kinetic parameters have been determined using the chromogenic analog o-nitrophenyl-beta-D ...
openaire +2 more sources
Purification and characterization of α-D-galactosidase from lactobacillus sporogenes
碩士本實驗以基因工程的方法,將稻米α-galactosidase基因接到質 體上(pLDHEB),並轉殖入乳酸菌(Lactobacillus sporogenes)表現出較多的α-半乳糖水解酵素。經由大量製備純化後予以定性和定量分析,並測試B型紅血球轉變為O型紅血球之情形。 在最佳條件下培養乳酸菌,並利用超音波細胞破碎後,1升的菌液可以得到1.2 g、40單位的粗製蛋白質。再由液相層析方法使用分子篩管柱層析(Sephadex G-150、Sephacryl S-100)、陰離子交換樹脂(DEAE ...
陳奕任; Chen, Yi-jen
core +1 more source
α-d-galactosidase deficiency in coconut endosperm: its possible pleiotropic effects in makapuno
α-d-galactosidase (α-d-galactoside galactohydrolase, EC 3.2.1.22) activity in the normal endosperm increased with age continually up to endosperm maturity.
Mendoza, Evelyn Mae T. +2 more
core
Anderson-Fabry: una malattia rara?
non ...
Paolo Colomba +11 more
doaj +1 more source
Acometimento renal na doença de Fabry
Resumo Todas as células do corpo humano apresentam acúmulo de globotriaosilceramida (Gb3) na doença de Fabry devido à mutação que ocorre no gene da enzima α-galactosidase A. Trata-se de uma doença ligada ao sexo.
Hugo Abensur, Marlene Antônia dos Reis
doaj +1 more source
α-D-Gal-cyclophellitol cyclosulfamidate is a Michaelis complex analog that stabilizes therapeutic lysosomal α-galactosidase A in Fabry disease [PDF]
Fabry disease is an inherited lysosomal storage disorder that is characterized by a deficiency in lysosomal α-D-galactosidase activity. One current therapeutic strategy involves enzyme replacement therapy, in which patients are treated with recombinant ...
Schaafa, Amanda +13 more
core +1 more source

