نتایج جستجو برای: acid maltase deficiency
تعداد نتایج: 868406 فیلتر نتایج به سال:
the effects of dietary vitamin e levels on mucosal maltase and alkaline phosphatase (alp) enzyme activities and on the amount of mucosal malonyldialdehyde (mda) in broiler chickens were studied in the present study. one hundred and eighty of day old male broiler chicks (ross 308 strain) were randomly assigned into five groups, each with three replicates and 12 chicks in each replicate. chickens...
We analyzed a 5,770-bp genomic region of Drosophila virilis that contains a cluster of two maltase genes showing sequence similarity with genes in a cluster of three maltase genes previously identified in Drosophila melanogaster. The D. virilis maltase genes are designated Mav1 and Mav2. In addition to being different in gene number, the cluster of genes in D. virilis differs dramatically in in...
Glycogen storage disease, type II (GSDII; Pompe disease; acid maltase deficiency) is an autosomal recessive disease caused by mutations of the GAA gene that lead to deficient acid alpha-glucosidase enzyme activity and accumulation of lysosomal glycogen. Although measurement of acid alpha-glucosidase enzyme activity in fibroblasts remains the gold standard for the diagnosis of GSDII, analysis of...
Thalassemia Intermedia; Folic Acid and Vitamin B12 Supplementation. What We Know and What is Needed?
This study presented a mini review on folic acid deficiency and recommendations for its supplementation in thalassemia intermedia (TI). TI is a clinical condition which lies between thalassemia major and thalassemia minor. Although TI patients may not need regular blood transfusion, precise diagnosis and management are critical for the prevention of clinical complications and quality of life im...
One of the most important carbohydrate-splitting enzymes is themaltase-glucoamylase which catalyzes the hydrolysis of alpha-glucosidic linkages. Maltase-glucoamylase inhibitors during the last few years have aroused medical interests in the treatment of diabetes. They contribute to a better understanding of the mechanism of maltase-glucoamylase. At present there are many different classes of ma...
In screening experiments for rat intestinal alpha-glucosidase (sucrase and maltase) inhibitors in 325 plants cultivated in Japan's southern island, of Tanegashima, marked inhibition against both sucrase and maltase was found in the extract of the fruit of Solanum torvum. Enzyme-assay guided fractionation of the extract led to the isolation of methyl caffeate (1) as a rat intestinal sucrase and ...
Regulation of MAL1+ gene expression encoding maltase in Schizosaccharomyces pombe by added inositol.
In this study, the effects of inositol addition on maltase activity and expression of MAL1+ gene encoding maltase in Schizosaccharomyces pombe were investigated. The maximum specific maltase activity was observed, when the concentration of inositol reached 6.0 microg/ml in the synthetic medium containing 2.0% glucose. At 1.0 microg/ml inositol concentration, the maltase activity continuously de...
Other potential modes of metabolic therapy include: 1.Supplementation of the missing compound, e.g. CoQ10 which is effective only in primary Q10 deficiency and is given to most patients with mitochondrial disorders even if deficiency is not tested; 2. Pharmacologically increasing the oxidative capacity of muscle (by giving various ‘cocktails’ of oxygen species scavengers that include: carnitine...
Inbred haploid strains of Saccharomyces cerevisiae carrying MAL1, MAL2 or MAL6 in a common background have been crossed to each other and to strains carrying no active MAL loci. The kinetics of maltase induction and the induced maltase levels have been examined in the inbred strains and in haploid segregants of the crosses. Differences have been found in the kinetics of induction and induced ma...
Pompe disease is a fatal genetic muscle disorder caused by a deficiency of acid alpha-glucosidase (GAA), a glycogen degrading lysosomal enzyme. GAA-deficient (AMD) Japanese quails exhibit progressive myopathy and cannot lift their wings, fly, or right themselves from the supine position (flip test). Six 4-wk-old acid maltase-deficient quails, with the clinical symptoms listed, were intravenousl...
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