Results 21 to 30 of about 1,158,721 (342)

Spatial regulation of alpha-galactosidase activity and its influence on raffinose family oligosaccharides during seed maturation and germination in Cicer arietinum. [PDF]

Plant Signal Behav, 2020
Alpha-galactosides or Raffinose Family Oligosaccharides (RFOs) are enriched in legumes and are considered as anti-nutritional factors responsible for inducing flatulence.
Arunraj R, Skori L, Kumar A, Hickerson NMN, Shoma N, M V, Samuel MA.   +6 more
europepmc   +2 more sources

Prediction of the responsiveness to pharmacological chaperones: lysosomal human alpha-galactosidase, a case of study

Orphanet Journal of Rare Diseases, 2010
Background The pharmacological chaperones therapy is a promising approach to cure genetic diseases. It relies on substrate competitors used at sub-inhibitory concentration which can be administered orally, reach difficult tissues and have low cost ...
Correra Antonella, Cammisa Marco, Guarracino Mario R, Andreotti Giuseppina, Cubellis Maria   +4 more
doaj   +2 more sources

Correlation between GLA variants and alpha-Galactosidase A profile in dried blood spot: an observational study in Brazilian patients. [PDF]

Orphanet J Rare Dis, 2020
Background Fabry disease is a rare X-linked inherited disorder caused by deficiency of α-Galactosidase A. Hundreds of mutations and non-coding haplotypes in the GLA gene have been described; however, many are variants of unknown significance, prompting ...
Varela P, Mastroianni Kirsztajn G, Motta FL, Martin RP, Turaça LT, Ferrer HLF, Gomes CP, Nicolicht P, Mara Marins M, Pessoa JG, Braga MC, D'Almeida V, Martins AM, Pesquero JB.   +13 more
europepmc   +2 more sources

Pegunigalsidase alfa: a novel, pegylated recombinant alpha-galactosidase enzyme for the treatment of Fabry disease. [PDF]

Front Genet
Fabry disease, a rare X-linked genetic disorder, results from pathogenic variants in GLA, leading to deficient lysosomal α-galactosidase A enzyme activity and multi-organ manifestations. Since 2001, enzyme replacement therapy (ERT), using agalsidase alfa
Germain DP, Linhart A.
europepmc   +2 more sources

Clinical impact of the alpha-galactosidase A gene single nucleotide polymorphism -10C>T: A single-center observational study. [PDF]

Medicine (Baltimore), 2018
Single nucleotide polymorphisms (SNPs) in the alpha-galactosidase A gene region (GLA) have been discussed as potential cause of symptoms and organ manifestations similarly to those seen in Fabry disease (FD).
Oder D, Liu D, Üçeyler N, Sommer C, Hu K, Salinger T, Müntze J, Petritsch B, Ertl G, Wanner C, Nordbeck P, Weidemann F.   +11 more
europepmc   +2 more sources

Case report of a 45-year old female Fabry disease patient carrying two alpha-galactosidase A gene mutation alleles. [PDF]

BMC Med Genet, 2016
Oder D, Vergho D, Ertl G, Wanner C, Nordbeck P.   +4 more
europepmc   +3 more sources

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, Jobudhas Jenisha, Pasupathi Rathinasabapathi, Rex Arunraj   +3 more
doaj   +1 more source

Biodegradation of sugarcane bagasse biomass using recombinant alpha-galactosidase overexpressing whole-cell <i>E.coli</i>: a sustainable method of agricultural waste utilization. [PDF]

3 Biotech
Vetriselvi PM, Narasimhan MK, Samuel M, Arunraj R.   +3 more
europepmc   +3 more sources

Studies on human liver alpha-galactosidases. II. Purification and enzymatic properties of alpha-galactosidase B (alpha-N-acetylgalactosaminidase).

Journal of Biological Chemistry, 1979
K J, Dean, C C, Sweeley
openaire   +3 more sources

Affective and cognitive behavior in the alpha-galactosidase A deficient mouse model of Fabry disease. [PDF]

PLoS One, 2017
Fabry disease is an X-linked inherited lysosomal storage disorder with intracellular accumulation of globotriaosylceramide (Gb3) due to α-galactosidase A (α-Gal A) deficiency.
Hofmann L, Karl F, Sommer C, Üçeyler N.   +3 more
europepmc   +2 more sources