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Blister
Immunocytochemical Identification and Localization of Active and Inactive alpha-Amylase and Pullulanase in Cells of Clostridium thermosulfurogenes EM1.
Carcinogenesis
Human DBR1 modulates the recycling of snRNPs to affect alternative RNA splicing and contributes to the suppression of cancer development.
Cardiomyopathies
Glycogen storage disease type III (glycogen debranching enzyme deficiency): correlation of biochemical defects with myopathy and cardiomyopathy.
Cardiomyopathies
Successful treatment of severe cardiomyopathy in glycogen storage disease type III with D,L-3-hydroxybutyrate, ketogenic and high protein diet.
Cardiomyopathy, Hypertrophic
A case of glycogen storage disease type III (glycogen debranching enzyme deficiency) with liver cirrhosis and hypertrophic cardiomyopathy.
Genetic Diseases, Inborn
[Biological and physiopathological aspects of hepatic glycogenoses]
Glycogen Storage Disease
A case of glycogen storage disease type III (glycogen debranching enzyme deficiency) with liver cirrhosis and hypertrophic cardiomyopathy.
Glycogen Storage Disease
Debranching enzyme in fibroblasts, amniotic fluid cells and chorionic villi: pre- and postnatal diagnosis of glycogenosis type III.
Glycogen Storage Disease
Definitive prenatal diagnosis for type III glycogen storage disease.
Glycogen Storage Disease
Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease type IIIa are phenotype-specific: An investigator-initiated, randomized, crossover study.
Glycogen Storage Disease
Glycogen debranching enzyme deficiency: long-term study of serum enzyme activities and clinical features.
Glycogen Storage Disease
Glycogen debranching enzyme: purification, antibody characterization, and immunoblot analyses of type III glycogen storage disease.
Glycogen Storage Disease
Glycogen storage disease type III (glycogen debranching enzyme deficiency): correlation of biochemical defects with myopathy and cardiomyopathy.
Glycogen Storage Disease
Heart Failure Due to Severe Hypertrophic Cardiomyopathy Reversed by Low Calorie, High Protein Dietary Adjustments in a Glycogen Storage Disease Type IIIa Patient.
Glycogen Storage Disease
Hexose and protein tolerance tests in children with liver glycogenosis caused by a deficiency of the debranching enzyme system.
Glycogen Storage Disease
Identification of a 5' splice junction mutation in the debranching enzyme gene in a Japanese patient with glycogen storage disease type IIIa.
Glycogen Storage Disease
Leukocyte debranching enzyme in glycogen storage disease.
Glycogen Storage Disease
Phenylketonuria and glycogen storage disease type III in sibs of one family.
Glycogen Storage Disease
Reversal of glycogen storage disease type IIIa-related cardiomyopathy with modification of diet.
Glycogen Storage Disease
Some properties of fibroblasts from a patient with debrancher deficiency.
Glycogen Storage Disease
Successful treatment of severe cardiomyopathy in glycogen storage disease type III with D,L-3-hydroxybutyrate, ketogenic and high protein diet.
Glycogen Storage Disease
Type IIIb glycogen storage disease associated with end-stage cirrhosis and hepatocellular carcinoma. The Liver Transplant Group.
Glycogen Storage Disease
Uniparental isodisomy of chromosome 1 results in glycogen storage disease type III with profound growth retardation.
Glycogen Storage Disease
[Biological and physiopathological aspects of hepatic glycogenoses]
Glycogen Storage Disease
[Debranching enzyme activity in leukocytes and glycogen content of erythrocytes in patients with type III glycogenosis]
Glycogen Storage Disease
[Genetic heterogeneity and the diagnosis of hepatic glycogenoses]
Glycogen Storage Disease Type III
A case of glycogen storage disease type III (glycogen debranching enzyme deficiency) with liver cirrhosis and hypertrophic cardiomyopathy.
Glycogen Storage Disease Type III
Glycogen debranching enzyme deficiency: long-term study of serum enzyme activities and clinical features.
Glycogen Storage Disease Type III
Glycogen storage disease type III (glycogen debranching enzyme deficiency): correlation of biochemical defects with myopathy and cardiomyopathy.
Glycogen Storage Disease Type III
Heart Failure Due to Severe Hypertrophic Cardiomyopathy Reversed by Low Calorie, High Protein Dietary Adjustments in a Glycogen Storage Disease Type IIIa Patient.
Glycogen Storage Disease Type III
Reversal of glycogen storage disease type IIIa-related cardiomyopathy with modification of diet.
Glycogen Storage Disease Type III
Successful treatment of severe cardiomyopathy in glycogen storage disease type III with D,L-3-hydroxybutyrate, ketogenic and high protein diet.
Glycogen Storage Disease Type III
Uniparental isodisomy of chromosome 1 results in glycogen storage disease type III with profound growth retardation.
Glycogen Storage Disease Type V
Glycogen debrancher deficiency is reproduced in muscle culture.
Hepatomegaly
Successful treatment of severe cardiomyopathy in glycogen storage disease type III with D,L-3-hydroxybutyrate, ketogenic and high protein diet.
Hypoglycemia
Successful treatment of severe cardiomyopathy in glycogen storage disease type III with D,L-3-hydroxybutyrate, ketogenic and high protein diet.
Infections
Conformational Changes in the 5' End of the HIV-1 Genome Dependent on the Debranching Enzyme DBR1 During Early Stages of Infection.
Infections
Fluorescent Branched RNAs for High-Throughput Analysis of Dbr1 Enzyme Kinetics and Inhibition.
Liver Cirrhosis
A case of glycogen storage disease type III (glycogen debranching enzyme deficiency) with liver cirrhosis and hypertrophic cardiomyopathy.
Meningoencephalitis
New immunodeficiency syndromes that help us understand the IFN-mediated antiviral immune response.
Muscular Diseases
Deep morphological analysis of muscle biopsies from type III glycogenesis (GSDIII), debranching enzyme deficiency, revealed stereotyped vacuolar myopathy and autophagy impairment.
Muscular Diseases
Glycogen storage disease type III (glycogen debranching enzyme deficiency): correlation of biochemical defects with myopathy and cardiomyopathy.
Muscular Diseases
Investigating glycogenosis type III patients with multi-parametric functional NMR imaging and spectroscopy.
Myopathies, Nemaline
[A 22-year-old man with long-standing weakness and atrophy predominantly in the lower extremities]
Neurodegenerative Diseases
Design, Synthesis, and Properties of Phosphoramidate 2',5'-Linked Branched RNA: Toward the Rational Design of Inhibitors of the RNA Lariat Debranching Enzyme.
Neurodegenerative Diseases
Fluorescent Branched RNAs for High-Throughput Analysis of Dbr1 Enzyme Kinetics and Inhibition.
Pemphigoid, Bullous
Differential display and cloning of the hippocampal gene mRNas in senescence accelerated mouse.
phosphorylase kinase deficiency
Myopathy due to glycogen storage disease: pathological and biochemical studies in relation to glycogenosome formation.
Pneumococcal Infections
Immune response to capsular polysaccharide and surface proteins of Streptococcus pneumoniae in patients with invasive pneumococcal disease.
Pneumonia
Characterization and expression of the structural gene for pullulanase, a maltose-inducible secreted protein of Klebsiella pneumoniae.
pullulanase deficiency
A Debranching Enzyme Deficiency in Endosperms of the Sugary-1 Mutants of Maize.
pullulanase deficiency
Debranching enzyme in fibroblasts, amniotic fluid cells and chorionic villi: pre- and postnatal diagnosis of glycogenosis type III.
pullulanase deficiency
Deep morphological analysis of muscle biopsies from type III glycogenesis (GSDIII), debranching enzyme deficiency, revealed stereotyped vacuolar myopathy and autophagy impairment.
pullulanase deficiency
Diet therapy in severe clinical expression of debrancher deficiency.
pullulanase deficiency
Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease type IIIa are phenotype-specific: An investigator-initiated, randomized, crossover study.
pullulanase deficiency
Glycogen storage disease type III (glycogen debranching enzyme deficiency): correlation of biochemical defects with myopathy and cardiomyopathy.
pullulanase deficiency
Investigating glycogenosis type III patients with multi-parametric functional NMR imaging and spectroscopy.
pullulanase deficiency
Phenylketonuria and glycogen storage disease type III in sibs of one family.
pullulanase deficiency
Successful treatment of severe cardiomyopathy in glycogen storage disease type III with D,L-3-hydroxybutyrate, ketogenic and high protein diet.
pullulanase deficiency
The molecular background of glycogen metabolism disorders.
pullulanase deficiency
Uniparental isodisomy of chromosome 1 results in glycogen storage disease type III with profound growth retardation.
pullulanase deficiency
[A 22-year-old man with long-standing weakness and atrophy predominantly in the lower extremities]
Spondylitis, Ankylosing
Molecular mimicry and ankylosing spondylitis: possible role of a novel sequence in pullulanase of Klebsiella pneumoniae.
Starvation
Differential proteome and cellular adhesion analyses of the probiotic bacterium Lactobacillus acidophilus NCFM grown on raffinose - an emerging prebiotic.
Starvation
Mucin- and carbohydrate-stimulated adhesion and subproteome changes of the probiotic bacterium Lactobacillus acidophilus NCFM.
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