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Thomas Hundsberger et al.
Journal of neurology, 260(9), 2279-2285 (2013-06-12)
Glycogen storage disease type II is a rare multi-systemic disorder characterised by an intracellular accumulation of glycogen due a mutation in the acid alpha glucosidase (GAA) gene. The level of residual enzyme activity, the genotype and other yet unknown factors
Sachin Talekar et al.
Bioresource technology, 147, 269-275 (2013-09-04)
A tri-enzyme biocatalyst "combi-CLEAs" with starch hydrolytic activity was prepared from commercially available alpha amylase, glucoamylase and pullulanase preparations by aggregating enzymes with ammonium sulphate followed by cross-linking formed aggregates for 4.5h with 40 mM glutaraldehyde. The effects of precipitant
A blood trans-alpha-glucosylase.
W H COPELAND et al.
Biochimica et biophysica acta, 22(1), 193-194 (1956-10-01)
Studies of lysosomal alpha-glucosidase. I. Purification and properties of the rat liver enzyme.
P L Jeffrey et al.
Biochemistry, 9(6), 1403-1415 (1970-03-17)
Tiziana Ventriglia et al.
Plant physiology, 148(1), 65-76 (2008-07-11)
ADP-glucose (Glc) pyrophosphorylase (ADP-Glc PPase) catalyzes the first committed step in starch biosynthesis. Higher plant ADP-Glc PPase is a heterotetramer (alpha(2)beta(2)) consisting of two small and two large subunits. There is increasing evidence that suggests that catalytic and regulatory properties
R.A. Rastall et al.
Biotechnology Letters, 13, 501-501 (1991)
Swati B Jadhav et al.
Carbohydrate polymers, 98(1), 1191-1197 (2013-08-31)
Two enzymes, α-amylase and glucoamylase have been individually and co-conjugated to pectin by covalent binding. Both the enzyme systems showed better thermal and pH stability over the free enzyme system with the complete retention of original activities. Mixture of individually
The maltase of Clostridium acetobutylicum; its specificity range and mode of action.
D FRENCH et al.
The Journal of biological chemistry, 187(2), 463-471 (1950-12-01)
Han-bing Zhang et al.
Zhonghua er ke za zhi = Chinese journal of pediatrics, 50(6), 415-419 (2012-08-31)
To analyze and summarize the characteristics of glycogen storage disease type II (Pompe disease) patients according to the clinical description and prognosis. Seventeen Chinese patients diagnosed by acid alpha-glucosidase (GAA) enzyme activity test were reviewed. Clinical data tables were designed.
Juan Antonio Tamayo-Ramos et al.
Biotechnology and bioengineering, 110(2), 543-551 (2012-09-06)
In filamentous fungi, most of the strategies used for the improvement of protein yields have been based on an increase in the transcript levels of a target gene. Strategies focusing at the translational level have been also described, but are
The subcellular distribution of enzymes in type II glycogenosis and the occurrence of an oligo-alpha-1,4-glucan glucohydrolase in human tissues.
B I Brown et al.
Biochimica et biophysica acta, 110(1), 124-133 (1965-10-25)
Zaki A El-Fiky et al.
Journal of food science, 77(6), M312-M317 (2012-05-16)
Baker's yeast, Saccharomyces cerevisiae, is a key component in bread baking. Total of 12 commercial baker's yeast and 2 hybrid strains were compared using traditional quality parameters. Total of 5 strains with high leavening power and the 2 hybrid strains
Shay-Whey M Koh et al.
Journal of neurochemistry, 109(3), 792-806 (2009-03-03)
The neuropeptide vasoactive intestinal peptide (VIP) is anti-inflammatory and protective in the immune and nervous systems, respectively. This study demonstrated in corneal endothelial (CE) cells injured by severe oxidative stress (1.4 mM H(2)O(2)) in bovine corneal organ cultures that VIP
Properties of human intestinal glucoamylase.
J J Kelly et al.
Biochimica et biophysica acta, 315(1), 113-122 (1973-07-05)
Ting-Ying Jiang et al.
PloS one, 7(7), e41131-e41131 (2012-07-21)
The N-terminal starch binding domain of Rhizopus oryzae glucoamylase (RoSBD) has a high binding affinity for raw starch. RoSBD has two ligand-binding sites, each containing a ligand-binding clamp: a polyN clamp residing near binding site I is unique in that
Abdelbagi M Ismail et al.
Annals of botany, 103(2), 197-209 (2008-11-13)
Flooding slows seed germination, imposes fatalities and delays seedling establishment in direct-seeded rice. This study describes responses of contrasting rice genotypes subjected to flooding or low oxygen stress during germination and discusses the basis of tolerance shown by certain cultivars.
Abdulameer E Al-Saffar et al.
Tropical animal health and production, 45(1), 327-334 (2012-07-31)
Two experiments were conducted to investigate the effect of phytase and/or multienzymes (protease, amyloglucoidase, xylanase, B-glucanase, cellulose, and hemicellulase) on improving the utilization of date pit (DP) in laying hens. In the first one, DP completely replaced corn in four
Wei Jiang Goh et al.
Langmuir : the ACS journal of surfaces and colloids, 28(49), 16864-16873 (2012-11-15)
Biofuels are fast advancing as a new research area to provide alternative sources of sustainable and clean energy. Recent advances in nanotechnology have sought to improve the efficiency of biofuel production, enhancing energy security. In this study, we have incorporated
M Kapelko et al.
Carbohydrate polymers, 97(2), 551-557 (2013-08-06)
The aim of the present study was to determine the impact of serial modifications of starch, including firstly starch extrusion or hydrolysis with pullulanase, followed by retrogradation (through freezing and defrosting of pastes) and acetylation (under industrial conditions), on its
Renata M Collares et al.
Journal of Zhejiang University. Science. B, 13(7), 579-586 (2012-07-05)
This work evaluates the enzymatic hydrolysis of starch from cassava using pectinase, α-amylase, and amyloglucosidase. A central composite rotational design (CCRD) was carried out to evaluate the effects of amyloglucosidase, pectinase, reaction time, and solid to liquid ratio. All the
Whitni K Redman et al.
Biofilm, 3, 100061-100061 (2021-11-27)
Novel anti-biofilm and dispersal agents are currently being investigated in an attempt to combat biofilm-associated wound infections. Glycoside hydrolases (GHs) are enzymes that hydrolyze the glycosidic bonds between sugars, such as those found within the exopolysaccharides of the biofilm matrix.
Min Jin Kwon et al.
BMC genomics, 13, 701-701 (2012-12-15)
Filamentous fungi such as Aspergillus niger are well known for their exceptionally high capacity for secretion of proteins, organic acids, and secondary metabolites and they are therefore used in biotechnology as versatile microbial production platforms. However, system-wide insights into their
John A Maga et al.
The Journal of biological chemistry, 288(3), 1428-1438 (2012-11-29)
We have used a peptide-based targeting system to improve lysosomal delivery of acid α-glucosidase (GAA), the enzyme deficient in patients with Pompe disease. Human GAA was fused to the glycosylation-independent lysosomal targeting (GILT) tag, which contains a portion of insulin-like
Cinthia Amiñoso et al.
Gene, 519(1), 169-172 (2013-02-14)
Pompe disease is an uncommon autosomal recessive glycogen storage disorder caused by deficiency of acid α-glucosidase. Classic infantile form triggers severe cardiomyopathy, hypotonia, and respiratory failure, leading to death within the first two years of life. The majority of patients
Ted J Langan et al.
Electrophoresis, 33(4), 607-613 (2012-03-28)
For the first time, a semi-permanent phospholipid coating is utilized in capillary electrophoresis-electrospray ionization-mass spectrometry (CE-ESI-MS). Although phospholipids in free solution are generally avoided in ESI, they did not interfere with the detection of linear and branched oligosaccharides using ESI
Incontinence in late-onset Pompe disease: an underdiagnosed treatable condition.
Gauthier Remiche et al.
European neurology, 68(2), 75-78 (2012-07-05)
Wanping Chen et al.
PloS one, 7(11), e49679-e49679 (2012-11-21)
Fungal amylolytic enzymes, including α-amylase, gluocoamylase and α-glucosidase, have been extensively exploited in diverse industrial applications such as high fructose syrup production, paper making, food processing and ethanol production. In this paper, amylolytic genes of 85 strains of fungi from
Livia Gómez et al.
Organic & biomolecular chemistry, 10(31), 6309-6321 (2012-06-28)
The synthesis, conformational study and inhibitory properties of diverse indolizidine and quinolizidine iminocyclitols are described. The compounds were chemo-enzymatically synthesized by two-step aldol addition and reductive amination reactions. The aldol addition of dihydroxyacetone phosphate (DHAP) to N-Cbz-piperidine carbaldehyde derivatives catalyzed
Frank Martiniuk et al.
Applied biochemistry and biotechnology, 171(4), 916-926 (2013-08-03)
Genetic deficiency of acid alpha glucosidase (GAA) results in glycogen storage disease type II (GSDII) or Pompe's disease. To investigate whether we could generate a functional recombinant human GAA enzyme (tobrhGAA) in tobacco seeds for future enzyme replacement therapy, we
Er James et al.
Applied microbiology and biotechnology, 96(2), 385-394 (2012-06-13)
This study demonstrates the potential of Aspergillus niger as a candidate expression system for virus-like particle production using gene fusion. Hepatitis B surface antigen (HBsAg) production, targeted through the secretory pathway in A. niger, resulted in completely assembled and properly
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