Results 1 to 10 of about 526,926 (248)
Bioconversion of Beet Molasses to Alpha-Galactosidase and Ethanol [PDF]
Frontiers in Microbiology, 2019 Molasses are sub-products of the sugar industry, rich in sucrose and containing other sugars like raffinose, glucose, and fructose. Alpha-galactosidases (EC. 3.2.1.22) catalyze the hydrolysis of alpha-(1,6) bonds of galactose residues in galacto-oligosaccharides (melibiose, raffinose, and stachyose) and complex galactomannans. Alpha-galactosidases have
Álvarez-Cao, María-Efigenia +3 more
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Alpha galactosidase A activity in Parkinson's disease [PDF]
Neurobiology of Disease, 2018 Glucocerebrosidase (GCase, deficient in Gaucher disease) enzymatic activity measured in dried blood spots of Parkinson's Disease (PD) cases is within healthy range but reduced compared to controls. It is not known whether activities of additional lysosomal enzymes are reduced in dried blood spots in PD.
R.N. Alcalay +20 more
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Neurobiology of Disease, 2018 The aberrant accumulation of alpha-synuclein (α-syn) is believed to contribute to the onset and pathogenesis of Parkinson's disease (PD). The autophagy-lysosome pathway (ALP) is responsible for the high capacity clearance of α-syn.
Michael P. Nelson +8 more
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Synthesis and characterization of a new fluorogenic substrate for alpha-galactosidase [PDF]
Analytical and Bioanalytical Chemistry, 2009 Alpha-galactosidase A hydrolyzes the terminal alpha-galactosyl moieties from glycolipids and glycoproteins in lysosomes. Mutations in alpha-galactosidase cause lysosomal accumulation of the glycosphingolipid, globotriaosylceramide, which leads to Fabry disease.
Zhen-Dan, Shi +8 more
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Molecular Pain, 2016 Background Fabry disease is an X-linked lysosomal storage disorder due to impaired activity of alpha-galactosidase A with intracellular accumulation of globotriaosylceramide. Associated small fiber pathology leads to characteristic pain in Fabry disease.
Nurcan Üçeyler MD +4 more
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Unexpectedly High Prevalence of Low Alpha-Galactosidase A Enzyme Activity in Patients with Focal Segmental Glomerulosclerosis [PDF]
Clinics, 2020 OBJECTIVES: Fabry disease (FD) is a rare disease associated with sphingolipid accumulation. Sphingolipids are components of plasma membranes that are important in podocyte function and accumulate in various glomerular diseases such as focal segmental ...
Nuri Baris Hasbal +8 more
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Fabry disease presenting with renal disease as the main manifestation diagnosed by renal biopsy: a case report [PDF]
Zhenduanxue lilun yu shijian, 2022 Fabry′s disease is an X-linked recessive genetic disease, which is mainly due to the mutation of (galactosidase A, GLA) gene. The decrease or loss of GLA activity eventually leads to the deposition of its metabolic substrate in multiple organs of
HAO Xu, WANG Weiming
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RIPK3 Contributes to Lyso-Gb3-Induced Podocyte Death
Cells, 2021 Fabry disease is a lysosomal storage disease with an X-linked heritage caused by absent or decreased activity of lysosomal enzymes named alpha-galactosidase A (α-gal A).
So-Young Kim +10 more
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Exploring the impact of 1-deoxynojirimycin on alpha-galactosidase activity and chickpea seed germination through in vitro experiments and molecular docking analysis [PDF]
Journal of Seed Science, 2023 : Seed germination is a tightly regulated physiological process. Hydrolytic enzymes provide energy that brings physiological, biochemical, and physical changes to the seed during germination.
Krishnamoorthy Vengatesh Prasanna +3 more
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Lentivirus-mediated gene therapy for Fabry disease
Nature Communications, 2021 Treatments for Fabry disease, an inherited lysosomal disorder caused by the deficiency of the enzyme alpha-galactosidase A, are not fully efficacious.
Aneal Khan +23 more
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