Results 1 to 10 of about 1,809,550 (296)

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   +3 more sources

Cloning and expression of a novel α-galactosidase from Lactobacillus amylolyticus L6 with hydrolytic and transgalactosyl properties. [PDF]

PLoS ONE, 2020
Lactobacillus amylolyticus L6, a gram-positive amylolytic bacterium isolated from naturally fermented tofu whey (NFTW), was able to hydrolyze raffinose and stachyose for the production of α-galactosidase. The cell-free extract of L.
Yongtao Fei, WenJuan Jiao, Ying Wang, Jinglong Liang, Gongliang Liu, Li Li   +5 more
doaj   +3 more sources

Valuation of agro-industrial wastes as substrates for heterologous production of α-galactosidase [PDF]

Microbial Cell Factories, 2018
Background The recycling of agro-industrial wastes is at present limited by the availability of efficient and low-cost enzyme cocktails. The use of these materials as culture media to produce the enzymes can contribute to the profitability of the ...
María-Efigenia Álvarez-Cao, Agustín Rico-Díaz, María-Esperanza Cerdán, Manuel Becerra, María-Isabel González-Siso   +4 more
doaj   +3 more sources

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

Misprocessing of α -Galactosidase A, Endoplasmic Reticulum Stress, and the Unfolded Protein Response. [PDF]

J Am Soc Nephrol
Visual Abstract Key Points The clinical significance of a number of missense variants of α-galactosidase A is often ambiguous. Defective proteostasis of some missense α-galactosidase A variants induced chronic endoplasmic reticulum stress and the ...
Živná M, Živná M, Dostálová G, Barešová V, Mušálková D, Svojšová K, Meiseles D, Kinstlinger S, Kuchař L, Asfaw B, Poupětová H, Vlášková H, Kmochová T, Vyleťal P, Hartmannová H, Hodaňová K, Stránecký V, Steiner-Mrázová L, Hnízda A, Živný J, Radina M, Votruba M, Sovová J, Trešlová H, Stolnaja L, Reková P, Roblová L, Honsová E, Rychlík I, Dvela-Levitt M, Bleyer AJ, Linhart A, Sikora J, Kmoch S.   +33 more
europepmc   +2 more sources

Cardiomyopathy associated with the Ala143Thr variant of the α-galactosidase A gene. [PDF]

Heart, 2020
Objective To investigate whether the Ala143Thr variant of the α-galactosidase A gene (A143T/GLA), with conflicting interpretations of pathogenicity, is associated with Fabry cardiomyopathy.
Valtola K, Nino-Quintero J, Hedman M, Lottonen-Raikaslehto L, Laitinen T, Maria M, Kantola I, Naukkarinen A, Laakso M, Kuusisto J.   +9 more
europepmc   +2 more sources

High-yield production and biochemical characterization of α-galactosidase produced from locally isolated Penicillium sp.

Bulletin of the National Research Centre, 2020
Background α-Galactosidase is widely used in various biotechnological applications such as food processing, beet sugar, the pulp and paper industries, synthesis of oligosaccharides by trans-galactosylation, hydraulic fracturing of oil and gas wells, and ...
Naganagouda Kote, A. C. Manjula, T. Vishwanatha, Aravind Gouda G. Patil   +3 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

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