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amylopectin/atrofi

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[Polysaccharide amylopectin-type storage myopathy].

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We report a late onset form of polysaccharide myopathy with progressive limb girdle muscles weakness, without cardiomyopathy. Muscle biopsy showed a vacuolar myopathy in type 1 fibres. The PAS positive diastase resistant deposits were made of filamentous material at electron microscopy similar to

Polysaccharide (amylopectin-like) storage myopathy histochemical ultrastructural and biochemical studies.

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A case of an adult polysaccharide myopathy is reported in a patient with progressive muscular atrophy and weakness of limb girdles. Histochemistry and electron microscopy showed in some muscle fibers, a storage material composed of amylopectin-like filaments. Biochemical results were normal and no
BACKGROUND It is becoming clear that ozone affects not only grain yield but also grain quality in rice. However, the biochemical mechanisms responsible for ozone-induced changes in appearance quality or components are poorly understood. We analyzed appearance quality and starch composition in the
The present study was undertaken to investigate the effect of the combination of soy protein, amylopectin, and chromium (SAC) on muscle protein synthesis and signal transduction pathways involved in protein synthesis (mTOR pathways, IGF-1, and AktSer473) and proteolysis (FOXO1Ser256; MURF1, MAFbx)
Relevant evidence of rice quality responses to increased temperatures under field condition has been obtained in our previous study. Understanding the intrinsic mechanism of increased temperatures and fertilizer treatment in regulating rice quality formation will be contributed to the development of
Gelation of starch is a process during short-term retrogradation. However, the long-term retrogradation always leads to the quality deterioration of starch-based food. In this work, a new type of modified tapioca starch (MTS) gel with maintained starch short-term gelation strength and retarded

Impact of frozen storage on whole wheat starch and its A-Type and B-Type granules isolated from frozen dough.

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The physicochemical properties of whole wheat starch and its A-Type and B-Type granules isolated from frozen dough at 0, 4 and 8 weeks of storage were investigated. Results showed that profiles of A-Type granules were more or less constant while significant differences of B-Type granules involving
Glycogen storage disease type IV (GSD-IV) is an autosomal recessive disease caused by a deficiency in glycogen-branching enzyme (GBE1) activity that results in the accumulation of amylopectin-like polysaccharide, which presumably leads to osmotic swelling and cell death. This disease is extremely

Staling white pan bread: fundamental causes.

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Staling, as it is applied to bakery foods, is a generic term covering a number of changes that occur in the products during normal storage. Consumers judge staleness by direct perception, which provides a subjective estimate that represents an unconscious integration of many factors. This review
In this research, for the first time, dual retrogradation-annealing modification was applied to three rice starches with 22.7%, 9.8% and 0.3% apparent amylose content (AAC). Amylose partially inhibited the effect of gelatinization on amylopectin crystallinity. Polymorphism in retrograded starches
Hardening issue in starch-based products that arises during storage, is ascribed to the long-term starch retrogradation which involves the recrystallisation of amylopectin. Present study aimed to delay storage hardening with the addition of high diastase honey bee honey (HBH) and low diastase
The staling of cakes enriched with untreated brans and endoxylanase-treated brans was evaluated by monitoring the changes in physicochemical, thermal, and sensorial properties of cakes during 7-d storage. Oat and rice bran were treated with different levels (0, 70, and 700 ppm) of an endoxylanase

Partial beta-amylolysis retards starch retrogradation in rice products.

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Starch retrogradation is the main cause of quality deterioration of starch-containing foods during storage. The current work investigated the effect of partial beta-amylolysis on the retrogradation of rice starch and the potential of beta-amylase in preparing rice products with extended shelf life.
A single 5-mg/kg oral dose of diclazuril affected both the asexual and sexual development of Eimeria tenella in experimentally inoculated chickens. In second-generation schizonts, early growth and nuclear divisions progressed normally, but a marked inhibition of merozoite formation was observed.

Glycogenosis with amylopectinoid deposits in a 13-year-old girl.

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We present a clinical study and muscle biopsy of a 13-year-old female who suffered hypertrophic cardiomyopathy, hepatosplenomegaly and myopathy of prolonged evolution. The muscle biopsy showed a glycogenosis with deposits of amylopectin-like material. Differential diagnosis was made with basophilic
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