Results 1 to 10 of about 28,650 (180)

Alpha galactosidase A activity in Parkinson's disease. [PDF]

Neurobiol Dis, 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. It is not known whether activities of additional lysosomal enzymes are reduced in dried blood spots in PD.
Alcalay RN, Wolf P, Levy OA, Kang UJ, Waters C, Fahn S, Ford B, Kuo SH, Vanegas N, Shah H, Liong C, Narayan S, Pauciulo MW, Nichols WC, Gan-Or Z, Rouleau GA, Chung WK, Oliva P, Keutzer J, Marder K, Zhang XK.   +20 more
europepmc   +6 more sources

Bioconversion of Beet Molasses to Alpha-Galactosidase and Ethanol. [PDF]

Front Microbiol, 2019
Molasses are sub-products of the sugar industry, rich in sucrose and containing other sugars like raffinose, glucose, and fructose. Alpha-galactosidases (EC. 3.2.1.22) catalyze the hydrolysis of alpha-(1,6) bonds of galactose residues in galacto-oligosaccharides (melibiose, raffinose, and stachyose) and complex galactomannans. Alpha-galactosidases have
Álvarez-Cao ME, Cerdán ME, González-Siso MI, Becerra M.   +3 more
europepmc   +7 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. More than 20% of the general population suffers from abdominal pain or discomfort caused by intestinal gas and by indigested or partially digested food residuals. Therefore, α-Gal is used in dietary
Fabris E, Bulfoni M, Nencioni A, Nencioni E.   +3 more
europepmc   +5 more sources

Alpha-Galactosidase A p.A143T, a non-Fabry disease-causing variant. [PDF]

Orphanet J Rare Dis, 2016
Background Fabry disease (FD) is an X-linked multisystemic disorder with a heterogeneous phenotype. Especially atypical or late-onset type 2 phenotypes present a therapeutical dilemma. Methods To determine the clinical impact of the alpha-Galactosidase A (GLA) p.A143T/ c.427G > A variation, we retrospectively analyzed 25 p.A143T patients in ...
Lenders M, Weidemann F, Kurschat C, Canaan-Kühl S, Duning T, Stypmann J, Schmitz B, Reiermann S, Krämer J, Blaschke D, Wanner C, Brand SM, Brand E.   +12 more
europepmc   +6 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

Sequence variations in the first exon of alpha-galactosidase A. [PDF]

Journal of Medical Genetics, 1993
The alpha-galactosidase A gene (GALA), which is deficient in males with Anderson-Fabry disease, is shown to be remarkably polymorphic in the 5' untranslated region. GALA contains seven exons. The first exon contains 60 bp of 5' untranslated sequence before the methionine initiation codon.
Sue Malcolm, Bryan Winchester, J P Davies   +2 more
openaire   +3 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). alpha-Galactosidase was induced by galactose, melibiose and raffinose, but galactose was the most efficient inducer.
Rezende, Sebastião Tavares de, Guimarães, Valéria Monteze, Rodrigues, Marília de Castro, Felix, Carlos Roberto   +3 more
openaire   +6 more sources

Synthesis and characterization of a new fluorogenic substrate for alpha-galactosidase [PDF]

Analytical and Bioanalytical Chemistry, 2009
Alpha-galactosidase A hydrolyzes the terminal alpha-galactosyl moieties from glycolipids and glycoproteins in lysosomes. Mutations in alpha-galactosidase cause lysosomal accumulation of the glycosphingolipid, globotriaosylceramide, which leads to Fabry disease.
Ke Liu, Ehud Goldin, Noel Southall, Christopher P. Austin, Omid Motabar, Zhen-Dan Shi, Ellen Sidransky, Gary L. Griffiths, Wei Zheng   +8 more
openaire   +3 more sources

.ALPHA.-Galactosidase of alkalophilic microorganisms. I. Identification and growth characteristics of .ALPHA.-galactosidase-producing microorganisms.

Agricultural and Biological Chemistry, 1976
Two kinds of α-galactosidase-producing microorganisms, strain No. 31–2 and strain No. 7–5, have been isolated from soil and subjected to a determinative study. On the basis of the morphological and physiological characters, the strain No. 31–2 was identified to be belonged to genus Micrococcus and the strain No. 7–5 to genus Bacillus. The former strain,
Koki Horikoshi, Teruhiko Akiba
openaire   +3 more sources

Alpha-galactosidase production and use in a hollow-fiber reactor [PDF]

Applied and Environmental Microbiology, 1976
Soybean milk serves as a base for a variety of beverages designed for consumption in developing countries. Soybean flour contains raffinose and stachyose considered to be responsible for flatulence often associated with these products (J.J. Rackis, D.H. Honig, D.J. Sessa, and F.R. Steggerda, 1970).
Karl L. Smiley, D. E. Hensley, Helen J. Gasdorf   +2 more
openaire   +3 more sources