Results 1 to 10 of about 57,322 (146)

Isolation and Identification of an α-Galactosidase-Producing Lactosphaera pasteurii Strain and Its Enzymatic Expression Analysis [PDF]

Molecules, 2022
α-Galactosidase (EC 3.2.1.22) refers to a group of enzymes that hydrolyze oligosaccharides containing α-galactoside-banded glycosides, such as stachyose, raffinose, and verbascose.
Yan Zhao, Jinghui Zhou, Shan Dai, Xiaozhu Liu, Xuewen Zhang   +4 more
doaj   +2 more sources

Reduced α-galactosidase A activity in zebrafish (Danio rerio) mirrors distinct features of Fabry nephropathy phenotype [PDF]

Molecular Genetics and Metabolism Reports, 2022
Fabry disease (FD) is a rare genetic lysosomal storage disorder, resulting from partial or complete lack of alpha-galactosidase A (α-GAL) enzyme, leading to systemic accumulation of substrate glycosphingolipids with a broad range of tissue damage ...
Hassan O.A. Elsaid, Jessica Furriol, Maria Blomqvist, Mette Diswall, Sabine Leh, Naouel Gharbi, Jan Haug Anonsen, Janka Babickova, Camilla Tøndel, Einar Svarstad, Hans-Peter Marti, Maximilian Krause   +11 more
doaj   +2 more sources

Systemic Treatment of Fabry Disease Using a Novel AAV9 Vector Expressing α-Galactosidase A [PDF]

Molecular Therapy: Methods & Clinical Development, 2021
Fabry disease is a rare X-linked disorder affecting α-galactosidase A, a rate-limiting enzyme in lysosomal catabolism of glycosphingolipids. Current treatments present important limitations, such as low half-life and limited distribution, which gene ...
Maria Grazia Biferi, Mathilde Cohen-Tannoudji, Andrea García-Silva, Olga Souto-Rodríguez, Irene Viéitez-González, Beatriz San-Millán-Tejado, Andrea Fernández-Carrera, Tania Pérez-Márquez, Susana Teijeira-Bautista, Soraya Barrera, Vanesa Domínguez, Thibaut Marais, África González-Fernández, Martine Barkats, Saida Ortolano   +14 more
doaj   +2 more sources

A statistical strategy for optimizing the production of α-galactosidase by a newly isolated Aspergillus niger NRC114 and assessing its efficacy in improving soymilk properties [PDF]

Journal of Genetic Engineering and Biotechnology, 2022
Background α-Galactosidase is widely distributed in plants, microorganisms, and animals, and it is produced by different fungal sources. Many studies have confirmed the valuable applications of α-galactosidase enzymes for various biotechnological ...
Ali M. Elshafei, Abdelmageed M. Othman, Maysa A. Elsayed, Gamil E. Ibrahim, Mohamed M. Hassan, Nayra S. Mehanna   +5 more
doaj   +2 more sources

Fabry disease: Identification of 50 novel α-galactosidase A mutations causing the classic phenotype and three-dimensional structural analysis of 29 missense mutations [PDF]

Human Genomics, 2006
Fabry disease, an X-linked recessive inborn error of glycosphingolipid catabolism, results from the deficient activity of the lysosomal exoglycohydrolase, α-galactosidase A (EC 3.2.1.22; α-Gal A).
Shabbeer Junaid, Yasuda Makiko, Benson Stacy D, Desnick Robert J   +3 more
doaj   +2 more sources

The New Pharmacological Chaperones PBXs Increase α-Galactosidase A Activity in Fabry Disease Cellular Models [PDF]

Biomolecules, 2021
Fabry disease is an X-linked multisystemic disorder caused by the impairment of lysosomal α-Galactosidase A, which leads to the progressive accumulation of glycosphingolipids and to defective lysosomal metabolism.
Pedro Besada, María Gallardo-Gómez, Tania Pérez-Márquez, Lucía Patiño-Álvarez, Sergio Pantano, Carlos Silva-López, Carmen Terán, Ana Arévalo-Gómez, Aurora Ruz-Zafra, Julián Fernández-Martín, Saida Ortolano   +10 more
doaj   +2 more sources

Carrier-Free Immobilization of α-Galactosidase as Nano-Biocatalysts for Synthesizing Prebiotic α-Galacto-Oligosaccharides [PDF]

Molecules, 2021
α-Galacto-oligosaccharides (α-GOSs) have great functions as prebiotics and therapeutics. This work established the method of batch synthesis of α-GOSs by immobilized α-galactosidase for the first time, laying a foundation for industrial applications in ...
Yan Liu, Jingyi Yang, Ke Wang, Feiyu Duan, Lili Lu   +4 more
doaj   +2 more sources

Optimizing human α-galactosidase for treatment of Fabry disease. [PDF]

Sci Rep, 2023
Hallows WC, Skvorak K, Agard N, Kruse N, Zhang X, Zhu Y, Botham RC, Chng C, Shukla C, Lao J, Miller M, Sero A, Viduya J, Ismaili MHA, McCluskie K, Schiffmann R, Silverman AP, Shen JS, Huisman GW.   +18 more
europepmc   +3 more sources

Detection of α-Galactosidase A Reaction in Samples Extracted from Dried Blood Spots Using Ion-Sensitive Field Effect Transistors [PDF]

Sensors
Fabry disease is a lysosomal storage disorder caused by a significant decrease in the activity or absence of the enzyme α-galactosidase A. The diagnostics of Fabry disease during newborn screening are reasonable, due to the availability of enzyme ...
Alexander Kuznetsov, Andrey Sheshil, Eugene Smolin, Vitaliy Grudtsov, Dmitriy Ryazantsev, Mark Shustinskiy, Tatiana Tikhonova, Irakli Kitiashvili, Valerii Vechorko, Natalia Komarova   +9 more
doaj   +2 more sources

Biochemical characterization and insights into the potency of the acidic Aspergillus niger NRC114 purified α-galactosidase in removing raffinose family oligosaccharides from soymilk yogurt [PDF]

BMC Biotechnology, 2023
Background Because humans lack α-galactosidase, foods containing certain oligosaccharides from the raffinose family, such as soybeans and other legumes, may disrupt digestion and cause flatulence.
Abdelmageed M. Othman, Ali M. Elshafei, Maysa A. Elsayed, Gamil E. Ibrahim, Mohamed M. Hassan, Nayra S. Mehanna   +5 more
doaj   +2 more sources