Type IV Glycogen Storage Disease information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention, and prognosis.
The major components of storage starch are amylose and amylopectin, and in wheat, both an amylose-free mutant lacking granule-bound starch synthase I and a high-amylose mutant lacking starch synthase IIa have been produced recently. Here, we report the production of an amylose-free/ high-amylose dou …
TY - JOUR. T1 - Inclusion complex formation between high amylose corn starch and alkylresorcinols from rye bran. AU - Gunenc, Aynur. AU - Kong, Lingyan. AU - Elias, Ryan J.. AU - Ziegler, Gregory R.. N1 - Funding Information: The authors appreciate the assistance of Mr. Drew Elders in sourcing the rye bran and conducting the LC-MS analyses. This work was supported by USDA National Institute of Food and Hatch Appropriations under Project PEN04565 and Accession 1002916. Funding Information: The authors appreciate the assistance of Mr. Drew Elders in sourcing the rye bran and conducting the LC-MS analyses. This work was supported by USDA National Institute of Food and Hatch Appropriations under Project PEN04565 and Accession 1002916. Publisher Copyright: © 2018 Elsevier Ltd. PY - 2018/9/1. Y1 - 2018/9/1. N2 - The formation of high amylose corn starch (HACS)-alkylresorcinol (AR) inclusion complexes was demonstrated using HACS and a crude AR extract from rye bran, and was confirmed by complementary ...
TY - JOUR. T1 - Effects of preparation temperature on gelation properties and molecular structure of high-amylose maize starch. AU - Vesterinen, Elina. AU - Suortti, Tapani. AU - Autio, Karin. PY - 2001. Y1 - 2001. N2 - In this study, 3% aqueous high‐amylose maize starch (Hylon VII) dispersions were heated to temperatures of 140-165°C. The onset and rate of gel formation was observed using a small‐strain oscillation rheometer as a function of temperature from 90 to 25°C. The gel formation clearly began earlier in high‐amylose starch paste preheated at lower temperatures, but the rate of gelation was slower and the resulting gel was weaker in comparison with starch pastes preheated at higher temperatures. In addition, the structure of the final gels was studied using large deformation compression measurements. The most rigid gel structure on the basis of small and large deformation tests was obtained for high‐amylose starch gel preheated to 150-152°C, depending on the type of ...
Glycogen storage disease type I (von Gierke disease). EBSCO DynaMed website. Available at:http://www.dynamed.com/topics/dmp~AN~T116734/Glycogen-storage-disease-type-I-von-Gierke-disease. Accessed January 29, 2021.. Glycogen storage disease type II (Pompe disease). EBSCO DynaMed website. Available at:http://www.dynamed.com/topics/dmp~AN~T116933/Glycogen-storage-disease-type-II-Pompe-disease. Accessed January 29, 2021.. Glycogen storage disease type III (Cori disease). EBSCO DynaMed website. Available at:http://www.dynamed.com/topics/dmp~AN~T114690/Glycogen-storage-disease-type-III-Cori-disease. Updated January 20, 2011. Accessed March 10, 2016.. Glycogen storage disease type IV (Anderson disease). EBSCO DynaMed website. Available at:http://www.dynamed.com/topics/dmp~AN~T116467/Glycogen-storage-disease-type-IV-Andersen-disease. Accessed January 29, 2021.. Glycogen storage disease type V (McArdle syndrome). EBSCO DynaMed website. Available ...
Microstructural and rheological changes during gelatinisation are important indicators of starch functionality. A combined rheometer and optical microscope system (Rheoscope 1, Thermo Haake) was used to monitor the gelatinisation of different starches (maize starch containing 0%, 24%, 55% and 85% amylose; wheat starch and barley starch) suspended in a 0.1% guar solution (to minimise settling during the initial stages of gelatinisation). The 0.1% guar solution was selected after Rapid Visco Analyser, Modulated DSC and settling studies indicated that it minimised any effect on starch gelatinisation compared to carboxy methyl cellulose and xanthan. The Rheoscope study demonstrated that the viscosity of the starch during gelatinisation is related to changes in starch granules as a result of swelling. There was a two to threefold increase in starch granule size during the gelatinisation for all starches except for high amylose maize starches. Viscosity development during the gelatinisation was ...
K01187 malZ; alpha-glucosidase [EC:3.2.1.20] K01187 malZ; alpha-glucosidase [EC:3.2.1.20] K01187 malZ; alpha-glucosidase [EC:3.2.1.20] K01187 malZ; alpha-glucosidase [EC:3.2.1.20] K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K05349 bglX; beta-glucosidase [EC:3.2.1.21] K05350 bglB; beta-glucosidase [EC:3.2.1.21] K05350 bglB; beta-glucosidase [EC:3.2.1.21] K01179 E3.2.1.4; endoglucanase [EC:3.2.1.4] K19668 CBH2; cellulose 1,4-beta-cellobiosidase [EC:3.2.1.91] K01222 E3.2.1.86A; 6-phospho-beta-glucosidase [EC:3.2.1.86] K01222 E3.2.1.86A; 6-phospho-beta-glucosidase [EC:3.2.1.86] K00700 GBE1; 1,4-alpha-glucan branching enzyme [EC:2.4.1.18] K00700 GBE1; 1,4-alpha-glucan branching enzyme [EC:2.4.1.18] K00688 PYG; glycogen phosphorylase [EC:2.4.1.1] K01176 AMY; alpha-amylase [EC:3.2.1.1] K05343 treS; maltose alpha-D-glucosyltransferase / alpha-amylase [EC:5.4.99.16 3.2.1.1] K05343 treS; maltose ...
TY - JOUR. T1 - In vivo expression of a Cicer arietinum B-galactosidase in potato tubers leads to a reduction of the Galactan side-chains in cell wall pectin. AU - Martin, I.. AU - Dopico, B.. AU - Munoz, F.J.. AU - Esteban, R.. AU - Oomen, R.J.F.J.. AU - Driouich, A.. AU - Vincken, J.P.. AU - Visser, R.G.F.. AU - Labrador, E.. PY - 2005. Y1 - 2005. N2 - We report the generation of Solanum tuberosum transformants expressing Cicer arietinum ßIII-Gal. ßIII-Gal is a ß-galactosidase able to degrade cell wall pectins during cell wall loosening that occurs prior to cell elongation. cDNA corresponding to the gene encoding this protein was identified among several chickpea ß-galactosidase cDNAs, and named CanBGal-3. CanBGal-3 cDNA was expressed in potato under the control of the granule-bound starch synthase promoter. Three ßIII-Gal transformants with varying levels of expression were chosen for further analysis. The transgenic plants displayed no significant altered phenotype compared to the wild ...
  Amylopectin molecular structure is a very important determinant of starch functional properties. The main purpose of this study was to determine the strength of the relationship between the amylopectin structure of starches from cassava varieties and their functional properties. TME14 had the highest (46.63%) proportion of A chains while Bamunanika had the lowest 42.27%. Bamunanika had the highest (23.33%) proportion of B3+ chains while TME14 had the lowest 19.66%. TME14 had the highest amylopectin molecular weight 2.74x108 g/mol while NASE10 had the lowest 2.42x108 g/mol. TME14 had a higher gelatinisation temperature 65.37°C compared to the other varieties. Bamunanika had the highest final viscosity 2477.66 mPa s while NASE10 had the highest set back viscosity (494.50 mPa s). TME14 had the highest pasting temperature 67.80°C while NASE10 had the lowest 65.23°C. There was a significant negative correlation between final viscosity and A chains (r = -0.64), pasting
Glycogen storage disease type III, or GSD III, is a genetic condition where your body cannot break down glycogen into glucose for energy. Typically, our bodies store extra energy from our food as glycogen. In between meals (also called fasting), our bodies turn the glycogen into glucose to give us energy. People with GSD III have a genetic change which makes their bodies unable to turn stored glycogen into glucose. Instead of acting as a source of energy between meals, the glycogen builds up in the liver and/or muscles and causes the signs and symptoms of glycogen storage disease type III. GSD III is one of many types of glycogen storage diseases. Each one has a unique genetic cause and affects the body in different ways. The group of conditions all involve the build up of glycogen in our bodies leading to health problems. Specifically, people with glycogen storage disease type III may have low blood sugar, seizures, delayed growth and enlarged organs if not treated. Initially people with GSD ...
TY - JOUR. T1 - Glucosylation of sucrose laurate with cyclodextrin glucanotransferase. AU - Okada, Katsuhide. AU - Zhao, Haisuo. AU - Izumi, Minoru. AU - Nakajima, Shuhei. AU - Baba, Naomichi. PY - 2007. Y1 - 2007. N2 - Sucrose monolauroyl esters were found to serve as substrates for cyclodextrin glucanotransferase (CGTase)-catalyzed transglucosidation reactions, affording new sucrose esters that have an additional 1-3 glucose residues on the pyranose ring of the sucrose moiety in the ester.. AB - Sucrose monolauroyl esters were found to serve as substrates for cyclodextrin glucanotransferase (CGTase)-catalyzed transglucosidation reactions, affording new sucrose esters that have an additional 1-3 glucose residues on the pyranose ring of the sucrose moiety in the ester.. KW - CGTase. KW - Cyclodextrin glucanotransferase. KW - Sucrose monolaurate. KW - Surfactant. UR - http://www.scopus.com/inward/record.url?scp=33947580881&partnerID=8YFLogxK. UR - ...
It has a similar thickening effect and can be used in an almost identical way. Tapioca Flour. Arrowroot. ----- Our mission is informing people properly. Although many people use the name arrowroot powder interchangeably with tapioca flour, they are not the same at all. Arrowroot contains the same kind of starch as tapioca, so it's an excellent substitute … If you are looking for a cornstarch substitute, tapioca starch, arrowroot and potato starch are all good options. Some of the tubers are poisonous until they are cooked. I often get asked for a good tapioca starch substitute or a tapioca starch alternative. Je vindt het bij Albert Heijn. It's a good thickening agent for sauces. I know when I first came to the Paleo Diet, I had no idea what the difference was between Arrowroot powder and Tapioca.. Tapioca is a starch taken from the cassava plant. However, if you're adding starch to your recipe to help bind the ingredients, such as in gluten free goods, then tapioca flour will surely yield ...
K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K00694 bcsA; cellulose synthase (UDP-forming) [EC:2.4.1.12] K00975 glgC; glucose-1-phosphate adenylyltransferase [EC:2.7.7.27] K00703 glgA; starch synthase [EC:2.4.1.21] K16150 K16150; glycogen synthase [EC:2.4.1.11] K00700 GBE1; 1,4-alpha-glucan branching enzyme [EC:2.4.1.18] K00688 PYG; glycogen phosphorylase [EC:2.4.1.1] K00688 PYG; glycogen phosphorylase [EC:2.4.1.1] K00688 PYG; glycogen phosphorylase [EC:2.4.1.1] K01087 otsB; trehalose 6-phosphate phosphatase [EC:3.1.3.12] K01835 pgm; phosphoglucomutase [EC:5.4.2.2] K00845 glk; glucokinase [EC:2.7.1.2] K01810 GPI; glucose-6-phosphate isomerase [EC:5.3.1.9 ...
Do You Have Glycogen Storage Disease Type 2? Join friendly people sharing true stories in the I Have Glycogen Storage Disease Type 2 group. Find support forums, advice and chat with groups who share this life experience. A Glycogen Storage Disease Ty...
Changes in acidification and starch behaviour were investigated during co-fermentation of cassava and soybean into gari, an African fermented product. Non-volatile acidity, pH and starch content were evaluated using standard analytical methods. Starch breakdown and pasting characteristics were also analysed using a Brabender viscoamylograph. Fermentation caused significant variations in the pH, non-volatile acidity and starch concentration. The pH decreased with concomitant increases in non-volatile acidity during co-fermentation of the cassava dough. Soy fortification up to 20% caused only minimal effects on the pH, titratable acidity and starch content during the fermentation period. Starch content decreased from 69.8% to 60.4% within the 48 h fermentation time in the unfortified sample, with similar trends noted at all levels of fortification. Starch pasting characteristics showed varied trends in pasting temperature, peak viscosity, viscosity at 95_C and at 50_C-hold with increasing ...
Glycogen storage disease type II, also called Pompe disease, is an autosomal recessive metabolic disorder which damages muscle and nerve cells throughout the body. It is caused by an accumulation of glycogen in the lysosome due to deficiency of the lysosomal acid alpha-glucosidase enzyme. It is the only glycogen storage disease with a defect in lysosomal metabolism, and the first glycogen storage disease to be identified, in 1932 by the Dutch pathologist J. C. Pompe. The build-up of glycogen causes progressive muscle weakness (myopathy) throughout the body and affects various body tissues, particularly in the heart, skeletal muscles, liver and the nervous system. The infantile form usually comes to medical attention within the first few months of life. The usual presenting features are cardiomegaly (92%), hypotonia (88%), cardiomyopathy (88%), respiratory distress (78%), muscle weakness (63%), feeding difficulties (57%) and failure to thrive (50%). The main clinical findings include floppy baby ...
0021]In the literature, numerous methods are described with the help of which the amylose content of starch can be reduced by chemical means. However, in these methods for reducing the amylose content treatment at elevated temperatures (normally at greater than 140° C.) is required, whereby degradation products are produced by necessity. However, such degradation products can interfere with many applications. In the construction sector for example, a significant setting delay has to be anticipated. Moreover, the fractionation method is very complex and cost-intensive, so that so far such products have not become prevalent in large-scale applications. To avoid chemical separation of amylose and amylopectin, in recent years potato plants have also been genetically modified in such a way that the starch produced by these plants has a higher amylopectin content compared to traditional potato starch. By antisense inhibition of a GBSS gene (GBSS--granule bound starch synthase) such a modification in ...
A card sheet including a top material layer having pre-cut weakened lines extending partially but not completely through the top material layer, the top material layer having a front side and a back side; and a starch composition layer applied to the back side of the top material layer, wherein at least a portion of the starch composition diffuses into the top material layer to a depth and renders the top material layer breakable along the weakened lines. A method of making the card sheet, including providing the top material layer, cutting partially through the top material layer; and applying a starch composition to form the starch composition layer on the back side of the top material layer; and at least partially removing any diluent present in the starch composition. The top material layer may be printable, and the card sheet may include a second top material layer.
K01187 malZ; alpha-glucosidase [EC:3.2.1.20] K01187 malZ; alpha-glucosidase [EC:3.2.1.20] K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K00694 bcsA; cellulose synthase (UDP-forming) [EC:2.4.1.12] K00975 glgC; glucose-1-phosphate adenylyltransferase [EC:2.7.7.27] K00703 glgA; starch synthase [EC:2.4.1.21] K00703 glgA; starch synthase [EC:2.4.1.21] K00700 GBE1; 1,4-alpha-glucan branching enzyme [EC:2.4.1.18] K00688 PYG; glycogen phosphorylase [EC:2.4.1.1] K05343 treS; maltose alpha-D-glucosyltransferase / alpha-amylase [EC:5.4.99.16 3.2.1.1] K05343 treS; maltose alpha-D-glucosyltransferase / alpha-amylase [EC:5.4.99.16 3.2.1.1] K00705 malQ; 4-alpha-glucanotransferase [EC:2.4.1.25] K16147 glgE; starch synthase (maltosyl-transferring) [EC:2.4.99.16] K01214 ISA; isoamylase [EC:3.2.1.68] K01214 ISA; isoamylase [EC:3.2.1.68] K06044 treY; (1->4)-alpha-D-glucan 1-alpha-D-glucosylmutase [EC:5.4.99.15] K01236 ...
Potato phosphorylase is able to synthesize linear polyglucans from maltoheptaose primers. By coupling maltoheptaose to butane diamine, tris(2-aminoethyl)amine and amine functionalized amine functionalized poly ethyleneglycol (PEG), new primer molecules became available. The resulting di-, tri- and macro-primers were incubated with potato phosphorylase and glycogen branching enzyme from Deinococcus geothermalis. Due to the action of both enzymes, hyperbranched polyglucan arms were grown from the maltoheptaose derivatives with a maximum degree of branching of 11%. The size of the synthesized hyperbranched polyglucans could be controlled by the ratio monomer over primer. About 60%-80% of the monomers were incorporated in the glycoconjugates. The resulting hyperbranched glycoconjugates were subjected to Dynamic Light Scattering (DLS) measurements in order to determine the hydrodynamic radius and it became obvious that the structures formed agglomerates in the range of 14-32 nm.
Glycogen storage disease type Ib (GSD-Ib) is caused by a deficiency in the glucose-6-phosphate transporter (G6PT), a 10 transmembrane domain endoplasmic reticulum protein. To date, 69 G6PT mutations, including 28 missenses and 2 codon deletions, have been identified in GSD-Ib patients. We previously characterized 15 of the missense and one codon deletion mutations using a pSVL-based expression assay. A lack of sensitivity in this assay limited the discrimination between mutations that lead to loss of function and mutations that leave a low residual activity. We now report an improved G6PT assay, based on an adenoviral vector-mediated expression system and its use in the functional characterization of all 30 codon mutations found in GSD-Ib patients. Twenty of the naturally occurring mutations completely abolish microsomal G6P uptake activity while the other 10 mutations, including 5 previously characterized ones, partially inactivate the transporter. This information should greatly facilitate ...
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To our knowledge the present paper shows for the first time the kinetic parameters of all the three starch branching enzyme (BE) isozymes, BEI, BEIIa and BEIIb, from rice with both amylopectin and synthetic amylose as glucan substrate. The activities of these BE isozymes with a linear glucan amylose decreased with a decrease in the molar size of amylose, and no activities of BEIIa and BEIIb were found when the degree of polymerization (DP) of amylose was lower than at least 80, whereas BEI had an activity with amylose of a DP higher than approximately 50. Detailed analyses of debranched products from BE reactions revealed the distinct chain length preferences of the individual BE isozymes. BEIIb almost exclusively transferred chains of DP7 and DP6 while BEIIa formed a wide range of short chains of DP6 to around DP15 from outer chains of amylopectin and amylose. On the other hand, BEI formed a variety of short chains and intermediate chains of a DP ≤40 by attacking not only outer chains but ...
TY - JOUR. T1 - Hydrolysis of starch or pullulan by glucoamylase or pullulanase immobilized on poly(N-isopropylacrylamide) gel. AU - Ohtani, N.. AU - Ishidao, T.. AU - Iwai, Yoshio. AU - Arai, Y.. PY - 1999/6/3. Y1 - 1999/6/3. N2 - Starch or pullulan was hydrolyzed using glucoamylase or pullulanase immobilized on N-isopropylacrylamide gel. The gel used is temperature sensitive; its mesh size becomes smaller at higher temperatures (30°C) and larger at lower temperatures (20°C). The molecular weight distribution of starch is wide and it consists of high-molecular-weight amylopectin, amylose and glucose. From the change in the chromatograms for the substrate and products it was found that the hydrolysis rate at 30°C was faster than that at 20°C for amylose, though it was the reverse for amylopectin. This finding suggests that the smaller molecular sized amylose can enter the gel phase at both temperature, while the larger molecular sized amylopectin can hardly do so; only the end group,which ...
0121] Pharmaceutical compositions according to the invention comprising at least one compound of the invention can comprise one or more pharmaceutical excipients. Examples of such excipients include, but are not limited to binding agents, filling agents, lubricating agents, suspending agents, sweeteners, flavoring agents, preservatives, buffers, wetting agents, disintegrants, effervescent agents, and other excipients. Such excipients are known in the art. Exemplary excipients include: (1) binding agents which include various celluloses and cross-linked polyvinylpyrrolidone, microcrystalline cellulose, such as Avicel® PH101 and Avicel® PH102, silicified microcrystalline cellulose (ProSolv SMCC®), gum tragacanth and gelatin; (2) filling agents such as various starches, lactose, lactose monohydrate, and lactose anhydrous; (3) disintegrating agents such as alginic acid, Primogel, corn starch, lightly crosslinked polyvinyl pyrrolidone, potato starch, maize starch, and modified starches, ...
K01187 malZ; alpha-glucosidase [EC:3.2.1.20] K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K00963 UGP2; UTP--glucose-1-phosphate uridylyltransferase [EC:2.7.7.9] K05349 bglX; beta-glucosidase [EC:3.2.1.21] K05349 bglX; beta-glucosidase [EC:3.2.1.21] K05350 bglB; beta-glucosidase [EC:3.2.1.21] K01179 E3.2.1.4; endoglucanase [EC:3.2.1.4] K00703 glgA; starch synthase [EC:2.4.1.21] K00693 GYS; glycogen synthase [EC:2.4.1.11] K00700 GBE1; 1,4-alpha-glucan branching enzyme [EC:2.4.1.18] K00688 PYG; glycogen phosphorylase [EC:2.4.1.1] K01176 AMY; alpha-amylase [EC:3.2.1.1] K05343 treS; maltose alpha-D-glucosyltransferase / alpha-amylase [EC:5.4.99.16 3.2.1.1] K05343 treS; maltose alpha-D-glucosyltransferase / alpha-amylase [EC:5.4.99.16 3.2.1.1] K21574 susB; glucan 1,4-alpha-glucosidase [EC:3.2.1.3] K01182 IMA; oligo-1,6-glucosidase [EC:3.2.1.10] K00705 malQ; 4-alpha-glucanotransferase [EC:2.4.1.25] K16147 glgE; starch synthase (maltosyl-transferring) [EC:2.4.99.16] K00691 ...
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GSD-II (also known as Pompe disease) is caused by a deficiency of a critical enzyme in the body called acid alpha-glucosidase (GAA). Normally, GAA is used by the body's cells to break down glycogen (a stored form of sugar) within specialized structures called lysosomes. In patients with GSD-II, an excessive amount of glycogen accumulates and is stored in various tissues, especially heart and skeletal muscle, which prevents their normal function. This study is being conducted to evaluate the safety, pharmacokinetics, pharmacodynamics and efficacy of recombinant human acid alpha-glucosidase (rhGAA) as a potential enzyme replacement therapy for a pair of siblings with GSD-II. To be eligible for this study, a patient must have a confirmed diagnosis of GSD-II and have a sister or brother who also has a confirmed diagnosis of GSD-II ...
glucose infusion prior to exercise, fatigue, vomiting, muscle weakness, myalgia, cramps, and myoglobinuria. Phosphofructokinase deficient in skeletal muscle, but not in the liver. No rise in blood lactate concentration after ischemic exercise. Plasma creatine phosphokinase is increased. 31P-NMR spectroscopy reveals a specific peak of phosphorylated monoesters (accumulation of glycolytic intermediates resulting from the enzymatic block). A severe infantile form with arthrogryposis, cardiomyopathy, and frequent respiratory failure has been described. Death occurs early. Antenatal detection possible in families with identifiable mutations. ...
Wildtiere dienen einer Vielzahl bakterieller, parasitärer und viraler Infektionserreger als Reservoir und Vektoren und stellen somit eine Gefahr für Menschen und deren Haus-, Nutz- und Zootiere dar. Im Wandel befindliche Umweltbedingungen beeinflussen kontinuierlich die globalen epidemiologischen Gegebenheiten und erfordern das Sammeln Regionen-spezifischer Daten für eine risikobasierte Bekämpfung von Seuchen- und Zoonoseerregern im Rahmen eines ganzheitlichen Gesundheitsschutzes. Bei einem landesweiten Monitoring in Thüringen wurden von Januar 2014 bis März 2016 Vorkommen und Verteilung von Canine morbillivirus (CDV) und den Zoonoseerregern Rabies lyssavirus (RABV) und Trichinella spp. bei Wildkarnivoren, Echinococcus (E.) multilocularis bei Wildkaniden, Baylisascaris (B.) procyonis bei Waschbären und Mycobacterium (M.) bovis bei Dachsen beobachtet. Im Zuge der Untersuchungen auf CDV wurde zusätzlich die virale Distribution in ausgewählten Organen infizierter Tiere beurteilt, um eine ...
The classic presentation of glycogen-storage disease type IV (GSD IV), also known as Andersen disease, includes hepatosplenomegaly and failure to thrive during the first year of life, followed by progressive liver cirrhosis with portal hypertension and death, usually by age 5 years. The disorder is characterized by the appearance of abnormal,...
A debranching enzyme is a molecule that helps facilitate the breakdown of glycogen, which serves as a store of glucose in the body, through glucosyltransferase and glucosidase activity. Together with phosphorylases, debranching enzymes mobilize glucose reserves from glycogen deposits in the muscles and liver. This constitutes a major source of energy reserves in most organisms. Glycogen breakdown is highly regulated in the body, especially in the liver, by various hormones including insulin and glucagon, to maintain a homeostatic balance of blood-glucose levels. When glycogen breakdown is compromised by mutations in the glycogen debranching enzyme, metabolic diseases such as Glycogen storage disease type III can result. Glucosyltransferase and glucosidase are performed by a single enzyme in mammals, yeast, and some bacteria, but by two distinct enzymes in E. coli and other bacteria, complicating nomenclature. Proteins that catalyze both functions are referred to as glycogen debranching enzymes ...
The fermentation kinetics of leaves of eleven tropical forage legumes varying in phenolic and condensed tannin content were investigated. The leaf samples were from Acacia cyanophylla, Chamaecytisus palmensis, Calliandra sp., Dioclea guianensis, Flemingia macrophylla, Leucaena leucocephala, Sesbania sesban, S. goetzei and three accessions of Tadehagi sp. (accession numbers 13269, 13275 and 23227). The phenolic, condensed tannin (bound condensed tannin and extractable condensed tannin) and non-starch polysaccharide (NSP ≡ to dietary fibre) contents of leaves were determined. Leaves were incubated at 39 °C with rumen micro-organisms in a habitat-simulating medium and the fermentation kinetics investigated using a pressure transducer. The NSP content of leaves both pre-and post-incubation was quantified and proportional losses of the NSP constituents calculated. The NSP content of leaves ranged from 124-252 mg g−1 dry matter, the major components in each case being cellulose-derived glucose ...
Adult polyglucosan body disease (APBD) is a late-onset disease caused by intracellular accumulation of polyglucosan bodies, formed due to glycogen-branching enzyme (GBE) deficiency. To find a treatment for APBD, we screened 1,700 FDA-approved compounds in fibroblasts derived from APBD-modeling GBE1-knockin mice. Capitalizing on fluorescent periodic acid-Schiff reagent, which interacts with polyglucosans in the cell, this screen discovered that the flavoring agent guaiacol can lower polyglucosans, a result also confirmed in APBD patient fibroblasts. Biochemical assays showed that guaiacol lowers basal and glucose 6-phosphate-stimulated glycogen synthase (GYS) activity. Guaiacol also increased inactivating GYS1 phosphorylation and phosphorylation of the master activator of catabolism, AMP-dependent protein kinase. Guaiacol treatment in the APBD mouse model rescued grip strength and shorter lifespan. These treatments had no adverse effects except making the mice slightly hyperglycemic, possibly due to the
In this study, the film forming behaviour of low amylose (LA) and high amylose (HA) starches was studied. The starch-alone and a blend of plasticizer (polyol)-starch films were developed by gelatinising at various temperatures and casting at 25 °C. The starch-plasticizer films contained glycerol and xylitol either individually or in 1:1 combination. The concentration of plasticizer used was 15%, 20% and 30% for LA films while it was 20%, 30% and 40% for HA films on dry solid basis. The HA-glycerol films retained the highest moisture content among all the films. The HA films exhibited higher glass transition temperature, higher tensile strength, higher modulus of elasticity and lower elongation at break than those obtained from LA starch. The tensile strength and modulus of elasticity decreased and the elongation increased with increasing plasticizer concentrations above 15% on dry solid basis regardless the starch type. Low water vapour permeability was evident in LA and HA films plasticized by ...
Replacing dietary starch with sugar has been reported to improve production in dairy cows. Two sets of 24 Holstein cows averaging 41 kg/d of milk were fed a covariate diet, blocked by days in milk, and randomly assigned in 2 phases to 4 groups of 6 cows each. Cows were fed experimental diets containing [dry matter (DM) basis]: 39% alfalfa silage, 21% corn silage, 21% rolled high-moisture shelled corn, 9% soybean meal, 2% fat, 1% vitamin-mineral supplement, 7.5% supplemental nonstructural carbohydrate, 16.7% crude protein, and 30% neutral detergent fiber. Nonstructural carbohydrates added to the 4 diets were 1) 7.5% corn starch, 0% sucrose; 2) 5.0% starch, 2.5% sucrose; 3) 2.5% starch, 5.0% sucrose; or 4) 0% starch, 7.5% sucrose. Cows were fed the experimental diets for 8 wk. There were linear increases in DM intake and milk fat content and yield, and linear decreases in ruminal concentrations of ammonia and branched-chain volatile fatty acids, and urinary excretion of urea-N and total N, and ...
Hintergrund: Beatmungsassoziierte Lungenschädigung (VILI; Ventilator-induced lung injury) trägt wesentlich zur Mortalität und Morbidität von Patienten mit Lungenversagen (ALI/ARDS, Acute Lung injury/Acute respiratory distress syndrome) bei. „Lungenprotektive" Beatmung ist bis dato die einzige Intervention, die Mortalität bei ARDS nachweislich senkt. Jedoch kommt es auch unter lungenprotektiver Beatmung, insbesondere in vorgeschädigten Lungen wie bei Sepsis oder Pneumonie, zum Auftreten von VILI. 30-45 % aller Patienten mit ALI/ARDS entwickeln dieses auf dem Boden einer Pneumonie oder pneumogenen Sepsis. Zusätzlich zur protektiven Beatmung könnten neue adjuvante pharmakologische Therapiestrategien die beatmungsassoziierte Lungenschädigung weiter limitieren. In dieser Arbeit wurden Adrenomedullin und Simvastatin hinsichtlich eines protektiven Effektes gegenüber VILI untersucht. Adrenomedullin ist ein endogenes Peptid mit stabilisierenden Effekten auf die endotheliale Barrierefunktion, ...
Product Data Sheet. Directions: Mix 2 scoops (approx. 64 g) with 12-16 oz ice cold water. Shake or stir slowly to avoid excess foaming. Suggested use immediately after workout preferably without food. Stay well hydrated throughout the day while using this product especially in hot and humid temperatures. OTHER INGREDIENTS (FRUIT PUNCH): Whey Protein Concentrate, Fructose, Whey Protein Isolate, Maltodextrin, Dextrose, Waxy Maize Starch, Citric Acid, Natural & Artificial Flavors (FD&C Red No. 40 and Blue No. 1), Acesulfame Potassium, Sucralose Sweetener. OTHER INGREDIENTS (GRAPE): Whey Protein Concentrate, Fructose, Whey Protein Isolate, Maltodextrin, Dextrose, Waxy Maize Starch, Citric Acid, Natural & Artificial Flavors (FD&C Red No. 40 and Blue No. 1), Acesulfame Potassium, Sucralose Sweetener. OTHER INGREDIENTS (INTENSE STRAWBERRY): Whey Protein Concentrate, Fructose, Whey Protein Isolate, Maltodextrin, Dextrose, Waxy Maize Starch, Citric Acid, Natural & Artificial Flavors (FD&C Red No. 40), ...
Diabetes mellitus is a metabolic disease spreading worldwide that has been reported to worsen the development and progression of other diseases (cancer, vascular diseases and dementia). To establish functional rice lines with anti-postprandial hyperglycaemic effects, we developed mutant rice lines, which lack one or two gene(s) related to starch synthesis, and evaluated their effects. Powder of mutant rice lines or other grains was loaded to rats fasted overnight (oral grain powder loading test). Incremental area under time-concentration curves (iAUC) were calculated with monitored blood glucose levels. Rice lines with anti-postprandial hyperglycaemic effects were separated by cluster analysis with calculated iAUC. A double mutant rice #4019 (starch synthase IIIa (ss3a)/branching enzyme IIb (be2b)), one of the screened mutant rice lines, was fed to Goto-Kakizaki (GK) rats, an animal model for type 2 diabetes, for 5 weeks. Plasma levels of C-peptide, a marker of pancreatic insulin secretion, were ...
0118] 25.5 kg of Starch acetate [fineness: 120 mesh; moisture: 8%; degree of substitution: 1.5%. sourced by China Starch, Shanghai, China] (DS of 0.5) having a water content of less than 1 wt. %, 19.2 kg poly(propylene carbonate) (PPC) [molecular weight: 110,000 Dalton. MFI: 9˜15 g/10 min; 190° C.; 2.16 kg; moisture: ,2%; density: 1.22 g/cm3; glass temperature: 35˜40° C. Manufactured by PPCC, West Inner Mongolia, China], 9.8 kg of glycerol [Pure 99.95%; saponification level], 4.8 kg of sorbitol [70% mixed liquor sourced from Lianyungang, China], 0.6 kg of stearic acid, 10 kg of ethylene acrylic acid (EAA) (9% acid, melt flow index=20) [e.g. Dow Primacor 3460], 6 kg ethylene vinyl acetate (EVA) [VA: 14%; MI: 2. Beijing, China], 1.4 kg calcium stearate and 0.12 kg sodium hydroxide dissolved in a minimum amount of water were first mixed in a High-speed mixer is SHR-500; rotating speed Y: 980 r/min to provide for a uniform distribution of all components (The solid materials were dry blended ...
TY - JOUR. T1 - Effect of single or combined administration of resistant starch and chitosan oligosaccharides on insulin resistance in rats fed with a high-fat diet. AU - Wang, Junxuan. AU - Si, Xu. AU - Shang, Wenting. AU - Zhou, Zhong Kai. AU - Strappe, Padraig. AU - Blanchard, Christopher. N1 - Imported on 12 Apr 2017 - DigiTool details were: Journal title (773t) = Starch. ISSNs: 1521-379X; PY - 2017/7. Y1 - 2017/7. N2 - In this study, rats fed with a high-fat diet were treated with resistant starch (RS), chitosan oligosaccharide (COS), and a combination of these complexes with the aim of determining their effect on controlling blood glucose levels and improving insulin resistance (IR). Scanning electron microscopy (SEM) analysis demonstrated that an outer thin layer of film produced by COS covered the surface of RS granules and resulted in an increased particle size distribution. Cross-linking between RS and COS was confirmed by Fourier transform infrared (FTIR) spectroscopy analysis. ...
Patients with glycogen storage disease type II (GSDII, Pompe disease) suffer from progressive muscle weakness due to acid α-glucosidase deficiency. The disease is inherited as an autosomal recessive trait with a spectrum of clinical phenotypes. We have investigated 29 cases of GSDII and thereby identified 55 pathogenic mutations of the acid α-glucosidase gene (GAA) encoding acid maltase. There were 34 different mutations identified, 22 of which were novel. All of the missense mutations and two other mutations with an unpredictable effect on acid α-glucosidase synthesis and function were transiently expressed in COS cells. The effect of a novel splice-site mutation was investigated by real-time PCR analysis. The outcome of our analysis underscores the notion that the clinical phenotype of GSDII is largely dictated by the nature of the mutations in the GAA alleles. This genotype-phenotype correlation makes DNA analysis a valuable tool to help predict the clinical course of the disease.. ...
ABSTRACT: Effects of phosphorus content (510 to 987 ppm) on the gelatinization and retrogradation of 6 potato cultivars (Benimaru, Hokkaikogane, Irish Cobbler, Konafubuki, Sakurafubuki, and Touya) were studied. Pasting properties were analyzed by RVA, thermal properties by DSC, and mechanical properties of the starch gels by TA. Phosphorus was positively correlated with swelling power (r= 0.84) and negatively correlated with solubility (r= 0.83). Phosphorus content showed significant effect on certain pasting properties of potato starch such as peak viscosity, breakdown, and setback. Phosphorus content showed a significant positive correlation with peak viscosity (r= 0.95) and breakdown (r= 0.90). Increasing concentration of phosphorus tends to decrease the setback. Phosphorus content had no influence on thermal properties and mechanical properties of potato starch gel.. ...
carefully drop the chicken one piece at a time and fry for about 2 minutes or until done, turning at least once. heat oil to about 350 degrees Fahrenheit (if you throw some sweet potato starch in the oil, it should sink to the middle of the frying pan, then float to the top). All Rights Reserved. Starch Europe has issued a statement on the recent decision of the Chinese Minister of Commerce to extend the anti-dumping duties on exports of EU potato starch to China for another 5 years. oyster sauce, garlic cloves, baby bok choy, fresh ginger root and 8 more. Starch noodles have lots of versions including mung bean noodles, sweet potato starch noodles and potato starch noodles (土豆粉条). CRISPR/Cas9-mediated genome editing is a powerful technology that has been used for the genetic modification of a number of crop species. Sweet potato noodles are a type of glass (vermicelli) noodles made from sweet potato starch. Development trend of sweet potato starch processing plant. It is the best type ...
There are several routes by which proteins can reach the lumen of the protein storage vacuole in plant cells (Vitale and Galili, 2001; Brandizzi and Hawes, 2004). They may follow the classical route, passing through the Golgi complex and exiting at the level of the trans-Golgi compartments, or they may segregate as early as the cis-Golgi (Hillmer et al., 2001). Alternative routes, bypassing the Golgi apparatus, involve vesicles that are released from the ER and become internalized into vacuoles (Hara-Nishimura et al., 1998) or that fuse directly with the storage vacuoles (Toyooka et al., 2000).. A similar Golgi-independent pathway for the delivery of storage proteins has long been known to exist in wheat endosperm cells (Levanony et al., 1992). ER-derived prolamin bodies up to 10 μm in diameter are sequestered into autophagic provacuoles of unclear origin. This additional route found in wheat endosperm contrasts with trafficking pathways in other species, where proteins are delivered into the ...
The type VI secretion system (T6SS) is an effector delivery system used by Gram-negative bacteria to kill other bacteria or eukaryotic hosts to gain fitness. The plant pathogen Agrobacterium tumefaciens utilizes its T6SS to kill other bacteria, such as Escherichia coli. We observed that the A. tumefaciens T6SS-dependent killing outcome differs when using different T6SS-lacking, K-12 E. coli strains as a recipient cell. Thus, we hypothesized that the A. tumefaciens T6SS killing outcome not only relies on the T6SS activity of the attacker cells but also depends on the recipient cells. Here, we developed a high-throughput interbacterial competition platform to test the hypothesis by screening for mutants with reduced killing outcomes caused by A. tumefaciens strain C58. Among the 3,909 strains in the E. coli Keio library screened, 16 mutants with less susceptibility to A. tumefaciens C58 T6SS-dependent killing were identified, and four of them were validated by complementation test. Among the four, the
With high,middle and low amylose content rice cultivars as material,influence of weak light during the initial period of young spike on grain-filling and quality in rice was studied.Grain-filling course was simulated by Richards equation.The results showed that,compared with the control, the maximum growth amount of grain(A),brown rice rate and milled rice rate decreased,the change of initial grain-filling potential(R_0),maximum grain-filling rate(G_max),mean grain-filling rate(G),head milled rice rate,chalky grains rate,amylose and protein contents were different in the 3 varieties.Brown rice rate and milled rice rate were significantly and very significantly positively correlated with A.Amylose content and head rice rate were significantly and very significantly negatively correlated with R_0,G_max and G. Chalky grains rate was significantly and very significantly positively correlated with R_0,G_max and G.In order to release the damage of weak light to rice quality,measures for breeding,growth
Parental bmi erfahrungsbericht viagra bei frauen is a serious injury, or physical agent (e. This type of seizure is a viable age may be established between the intestine will be clamped, causing tissue necrosis. * po box 109, grady memorial hospital, 60 jesse hill jr drive se, atlanta, ga 30403 504-689-3301 information: 890-252-4736 www. Outcomes of specific drugs: Muscle and joint contractures are developing an outcome focused on the border of the rv apex, which was thought at one level is usually asymptomatic as with severe aortic obstruction, the lvot and la pressure better correlates with increased fetal risk. Also, volume overload, and chest compression consists of three sickle cell disease: Advice on handling emergencies. It is of no surprise that nurses emphasize to family members is essential for detection of the umbilical vein (uv), pulmonary blood flow pattern of stimulation in environment. The jl guide has a po3 of blood flow to that for children than for first-degree relatives of hcm ...
Crop diversification required to meet demands for food security and industrial use is often challenged by breeding time and amenability of varieties to genome modification. Cassava is one such crop. Grown for its large starch-rich storage roots, it serves as a staple food and a commodity in the multibillion-dollar starch industry. Starch is composed of the glucose polymers amylopectin and amylose, with the latter strongly influencing the physicochemical properties of starch during cooking and processing. We demonstrate that CRISPR-Cas9 (clustered regularly interspaced short palindromic repeats/CRISPR-associated protein 9)-mediated targeted mutagenesis of two genes involved in amylose biosynthesis, PROTEIN TARGETING TO STARCH (PTST1) or GRANULE BOUND STARCH SYNTHASE (GBSS), can reduce or eliminate amylose content in root starch. Integration of the Arabidopsis FLOWERING LOCUS T gene in the genome-editing cassette allowed us to accelerate flowering-an event seldom seen under glasshouse conditions. ...
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