Results 1 to 10 of about 1,158,721 (342)

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, P. Wolf, O.A. Levy, U.J. Kang, C. Waters, S. Fahn, B. Ford, S.H. Kuo, N. Vanegas, H. Shah, C. Liong, S. Narayan, M.W. Pauciulo, W.C. Nichols, Z. Gan-Or, G.A. Rouleau, W.K. Chung, P. Oliva, J. Keutzer, K. Marder, X.K. Zhang   +20 more
doaj   +6 more sources

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-Efigenia Álvarez-Cao, María-Esperanza Cerdán, María-Isabel González-Siso, Manuel Becerra   +3 more
doaj   +7 more sources

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.
Michael P. Nelson, Michel Boutin, Tonia E. Tse, Hailin Lu, Emily D. Haley, Xiaosen Ouyang, Jianhua Zhang, Christiane Auray-Blais, John J. Shacka   +8 more
doaj   +4 more sources

High throughput screening for inhibitors of alpha-galactosidase. [PDF]

Curr Chem Genomics, 2010
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 O, Liu K, Southall N, Marugan JJ, Goldin E, Sidransky E, Zheng W.   +6 more
europepmc   +5 more sources

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, Kazuki Nawashiro, Reiko Ohdake, Harutsugu Tatebe, Sayuri Shima, Akihiro Ueda, Junichiro Yoshimoto, Mizuki Ito, Takahiko Tokuda, Tatsuro Mutoh, Hirohisa Watanabe   +10 more
doaj   +2 more sources

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, Toru Harada, Eiko Fukuro, Masahisa Kobayashi, Toya Ohashi, Yoshikatsu Eto   +5 more
doaj   +2 more sources

Intra-Laboratory Validation of Alpha-Galactosidase Activity Measurement in Dietary Supplements [PDF]

Molecules, 2021
Introduction: Alpha-galactosidase (α-Gal) is an enzyme responsible for the hydrolyzation of glycolipids and glycoprotein commonly found in dietary sources.
Elena Fabris, Michela Bulfoni, Alessandro Nencioni, Emanuele Nencioni   +3 more
doaj   +2 more sources

Flow Cytometry-Based Assay to Detect Alpha Galactosidase Enzymatic Activity at the Cellular Level [PDF]

Cells
Background: Fabry disease is a progressive, X chromosome-linked lysosomal storage disorder with multiple organ dysfunction. Due to the absence or reduced activity of alpha-galactosidase A (AGAL), glycosphingolipids, primarily globotriaosyl-ceramide (Gb3),
Nóra Fekete, Luca Kamilla Li, Gergely Tibor Kozma, György Fekete, Éva Pállinger, Árpád Ferenc Kovács   +5 more
doaj   +2 more sources

Purification and characterization of an alpha-galactosidase from Aspergillus fumigatus [PDF]

Brazilian Archives of Biology and Technology, 2005
Aspergillus fumigatus secreted invertase (beta-fructofuranosidase) and alpha-galactosidase enzymatic activities able to hydrolyzing raffinose oligosaccharides (RO).
Sebastião Tavares de Rezende, Valéria Monteze Guimarães, Marília de Castro Rodrigues, Carlos Roberto Felix   +3 more
doaj   +2 more sources

Reference values for lysosomal enzymes activities using dried blood spots samples - a Brazilian experience [PDF]

Diagnostic Pathology, 2010
Background Lysosomal storage diseases (LSD) are inherited disorders caused by deficiency of lysosomal enzymes in which early diagnosis is essential to provide timely treatment. This study reports interval values for the activity of lysosomal enzymes that
Martins Ana M, Pereira Vanessa G, Rodrigues Mayra DB, Müller Karen B, D'Almeida Vânia   +4 more
doaj   +4 more sources