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.
María-Isabel González-Siso +2 more
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The lysosomal enzyme alpha-Galactosidase A is deficient in Parkinson's disease brain in association with the pathologic accumulation of alpha-synuclein [PDF]
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.
Michel Boutin +2 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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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.
R.N. Alcalay +20 more
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Enzymatic properties and clinical associations of serum alpha‐galactosidase A in Parkinson's disease [PDF]
Annals of Clinical and Translational Neurology, 2023 Objective Recent studies have revealed an association between Parkinson's disease (PD) and Fabry disease, a lysosomal storage disorder; however, the underlying mechanisms remain to be elucidated.
Yasuaki Mizutani +10 more
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Massive accumulation of globotriaosylceramide in various tissues from a Fabry patient with a high antibody titer against alpha-galactosidase A after 6 years of enzyme replacement therapy [PDF]
Molecular Genetics and Metabolism Reports, 2020 Fabry disease is an X-linked metabolic disorder due to a pathogenic mutation of the GLA gene. The accumulation of globotriaosylceramide (Gb3) damages multiple organs, including the heart, kidney and nervous system, especially in classical type Fabry ...
Kenichi Hongo +5 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 +2 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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