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A ribozyme selected from variants of U6 snRNA promotes 2 ',5 '-branch formation
RNA-A PUBLICATION OF THE RNA SOCIETY
Molecular genetic basis and prevalence of glycogen storage disease type IIIA in the Faroe Islands
EUROPEAN JOURNAL OF HUMAN GENETICS
Cloning, expression, and complementation test of the RNA lariat debranching enzyme cDNA from mouse
MOLECULES AND CELLS
Cloning and nucleotide sequence of the pullulanase gene of Thermus thermophilus HB8 and production of the enzyme in Escherichia coli
BIOSCIENCE BIOTECHNOLOGY AND BIOCHEMISTRY
Thermal blockage of viruslike particle formation for the yeast retrotransposon Ty3 reveals differences in the cellular stress response
ARCHIVES OF VIROLOGY
Reserve carbohydrates metabolism in the yeast Saccharomyces cerevisiae
FEMS MICROBIOLOGY REVIEWS
Activation of spinach pullulanase by reduction results in a decrease in the number of isomeric forms
BIOCHIMICA ET BIOPHYSICA ACTA-PROTEIN STRUCTURE AND MOLECULAR ENZYMOLOGY
Muscle glycogenoses
MUSCLE & NERVE
Novel exon 11 skipping mutation in a patient with glycogen storage diseasetype IIId
JOURNAL OF INHERITED METABOLIC DISEASE
Two loci control phytoglycogen production in the monocellular green alga Chlamydomonas reinhardtii
PLANT PHYSIOLOGY
Glycogen debranching enzyme in bovine brain
JOURNAL OF BIOCHEMISTRY
Wheat starch biosynthesis
EUPHYTICA
Time-course changes of the fine structure of rice starch grains during cooking process by scanning electron microscopy
JAPANESE JOURNAL OF CROP SCIENCE
Genotype-phenotype correlation in two frequent mutations and mutation update in type III glycogen storage disease
MOLECULAR GENETICS AND METABOLISM
The molecular diagnosis of metabolic myopathies
NEUROLOGIC CLINICS
Acid maltase deficiency and related myopathies
NEUROLOGIC CLINICS
The Saccharomyces cerevisiae YPR184w gene encodes the glycogen debranchingenzyme
FEMS MICROBIOLOGY LETTERS
Identification of a novel element required for processing of intron-encoded box C/D small nucleolar RNAs in Saccharomyces cerevisiae
MOLECULAR AND CELLULAR BIOLOGY
Hepatocellular carcinoma complicating liver cirrhosis in type IIIa glycogen storage disease
JOURNAL OF CLINICAL GASTROENTEROLOGY
The debranching enzyme complex missing in glycogen accumulating mutants ofChlamydomonas reinhardtii displays an isoamylase-type specificity
PLANT SCIENCE
Cloning and nucleotide sequence of a gene encoding a glycogen debranching enzyme in the trehalose operon from Arthrobacter sp Q36
BIOCHIMICA ET BIOPHYSICA ACTA-PROTEIN STRUCTURE AND MOLECULAR ENZYMOLOGY
Compound heterozygous patient with glycogen storage disease type III: Identification of two novel AGL mutations, a donor splice site mutation of Chinese origin and a 1-bp deletion of Japanese origin
AMERICAN JOURNAL OF MEDICAL GENETICS
Novel mutations in two Japanese cases of glycogen storage disease type IIIa and a review of the literature of the molecular basis of glycogen storagedisease type III
JOURNAL OF INHERITED METABOLIC DISEASE
A fresh understanding of the stereochemical behavior of glycosylases: Structural distinction of "inverting" (2-MCO-type) versus "retaining" (1-MCO-type) enzymes
ADVANCES IN CARBOHYDRATE CHEMISTRY AND BIOCHEMISTRY, VOL 55
Recent progress toward understanding biosynthesis of the amylopectin crystal
PLANT PHYSIOLOGY
Purification and characterization of an endoamylase from tulip (Tulipa gesneriana) bulbs
PHYSIOLOGIA PLANTARUM
Glycosyl fluorides in enzymatic reactions
CARBOHYDRATE RESEARCH
A polymorphic microsatellite in the limit dextrinase gene of barley (Hordeum vulgare L.)
MOLECULAR BREEDING
Making starch
CURRENT OPINION IN PLANT BIOLOGY
The stability and fate of a spliced intron from vertebrate cells
RNA-A PUBLICATION OF THE RNA SOCIETY
Genome-wide bioinformatic and molecular analysis of introns in Saccharomyces cerevisiae
RNA-A PUBLICATION OF THE RNA SOCIETY
Glycogen storage diseases - Phenotypic, genetic, and biochemical characteristics, and therapy
ENDOCRINOLOGY AND METABOLISM CLINICS OF NORTH AMERICA
The molecular background of glycogen metabolism disorders
JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM
The functions of 4-alpha-glucanotransferases and their use for the production of cyclic glucans
BIOTECHNOLOGY AND GENETIC ENGINEERING REVIEWS, VOL 16
Synthesis and kinetic evaluation of 4-deoxymaltopentaose and 4-deoxymaltohexaose as inhibitors of muscle and potato alpha-glucan phosphorylases
BIOCHEMICAL JOURNAL
Analyses of isoamylase gene activity in wild-type barley indicate its involvement in starch synthesis
PLANT MOLECULAR BIOLOGY
Novel donor splice site mutations of AGL gene in glycogen storage disease type IIIa
JOURNAL OF INHERITED METABOLIC DISEASE
Purification, characterization, and cDNA structure of isoamylase from developing endosperm of rice
PLANTA
A single limit dextrinase gene is expressed both in the developing endosperm and in germinated grains of barley
PLANT PHYSIOLOGY
ALKALINE DETERGENT ENZYMES FROM ALKALIPHILES - ENZYMATIC-PROPERTIES, GENETICS, AND STRUCTURES
Extremophiles
GLYCOGEN-STORAGE DISEASES AND THE LIVER
Bailliere's clinical gastroenterology
Fatal liver cirrhosis and esophageal variceal hemorrhage in a patient withtype IIIa glycogen storage disease
INTERNAL MEDICINE
CASE-REPORT - RUPTURE OF A GASTRIC VARIX IN LIVER-CIRRHOSIS ASSOCIATED WITH GLYCOGEN-STORAGE-DISEASE TYPE-III
Journal of gastroenterology and hepatology
SUCCESSFUL PREGNANCY IN A PATIENT WITH TYPE-III GLYCOGEN-STORAGE-DISEASE MANAGED WITH CORNSTARCH SUPPLEMENTS
British journal of obstetrics and gynaecology
PROTEIN HETEROGENEITY OF SPINACH PULLULANASE RESULTS FROM THE COEXISTENCE OF INTERCONVERTIBLE ISOMERIC FORMS OF THE MONOMERIC ENZYME
Biochemical journal
CDNA SEQUENCE AND HETEROLOGOUS EXPRESSION OF MONOMERIC SPINACH PULLULANASE - MULTIPLE ISOMERIC FORMS ARISE FROM THE SAME POLYPEPTIDE
Biochemical journal
SELECTION IN-VITRO OF NOVEL RIBOZYMES FROM A PARTIALLY RANDOMIZED U2 AND U6 SNRNA LIBRARY
EMBO journal
PRENATAL-DIAGNOSIS AND CARRIER DETECTION FOR GLYCOGEN-STORAGE-DISEASETYPE-III USING POLYMORPHIC DNA MARKERS
Prenatal diagnosis
GENOMIC DNA-SEQUENCE OF A RICE GENE CODING FOR A PULLULANASE-TYPE OF STARCH DEBRANCHING ENZYME
Biochimica et biophysica acta. Protein structure and molecular enzymology
DIVERGENT SOLID-PHASE SYNTHESIS OF NUCLEIC-ACID DENDRIMERS
Tetrahedron letters
CHARACTERIZATION OF SU1 ISOAMYLASE, A DETERMINANT OF STORAGE STARCH STRUCTURE IN MAIZE
Plant physiology
AN ASSAY-METHOD FOR GLYCOGEN DEBRANCHING ENZYME USING NEW FLUOROGENICSUBSTRATES AND ITS APPLICATION TO DETECTION OF THE ENZYME IN MOUSE-BRAIN
Journal of Biochemistry
LARGE-SCALE PURIFICATION AND CHARACTERIZATION OF BARLEY LIMIT DEXTRINASE, A MEMBER OF THE ALPHA-AMYLASE STRUCTURAL FAMILY
Cereal chemistry
REGIOSPECIFIC SOLID-PHASE SYNTHESIS OF BRANCHED OLIGONUCLEOTIDES - EFFECT OF VICINAL 2',5'-PHOSPHODIESTER (OR 2',3'-PHOSPHODIESTER) AND 3',5'-PHOSPHODIESTER LINKAGES ON THE FORMATION OF HAIRPIN DNA
Bioconjugate chemistry
A SINGLE-BASE DELETION IN THE 3'-CODING REGION OF GLYCOGEN-DEBRANCHING ENZYME IS PREVALENT IN GLYCOGEN-STORAGE-DISEASE TYPE IIIA IN A POPULATION OF NORTH-AFRICAN JEWISH PATIENTS
European journal of human genetics
AMINO-ACID-RESIDUES SPECIFIC FOR THE CATALYTIC ACTION TOWARDS ALPHA-1,6-GLUCOSIDIC LINKAGES IN KLEBSIELLA PULLULANASE
Journal of fermentation and bioengineering
ISOLATION AND NUCLEOTIDE-SEQUENCE OF HUMAN LIVER-GLYCOGEN DEBRANCHINGENZYME MESSENGER-RNA - IDENTIFICATION OF MULTIPLE TISSUE-SPECIFIC ISOFORMS
Gene
TYPE IIIB GLYCOGEN-STORAGE-DISEASE ASSOCIATED WITH END-STAGE CIRRHOSIS AND HEPATOCELLULAR-CARCINOMA
Hepatology
SEVERE GROWTH DEFECT IN A SCHIZOSACCHAROMYCES-POMBE MUTANT DEFECTIVE IN INTRON LARIAT DEGRADATION
Molecular and cellular biology
PULLULANASE IN MUNG BEAN COTYLEDONS - PURIFICATION, SOME PROPERTIES AND DEVELOPMENTAL PATTERN DURING AND FOLLOWING GERMINATION
Physiologia Plantarum
SELECTION OF A NONCONSENSUS BRANCH POINT IS INFLUENCED BY AN RNA STEM-LOOP STRUCTURE AND IS IMPORTANT TO CONFER STABILITY TO THE HERPES-SIMPLEX VIRUS 2-KILOBASE LATENCY-ASSOCIATED TRANSCRIPT
Journal of virology
ANALYSIS OF THE 2-KILOBASE LATENCY-ASSOCIATED TRANSCRIPT EXPRESSED INPC12 CELLS PRODUCTIVELY INFECTED WITH HERPES-SIMPLEX VIRUS TYPE-1 - EVIDENCE FOR A STABLE, NONLINEAR STRUCTURE
Journal of virology
POLYMORPHIC MARKERS OF THE GLYCOGEN DEBRANCHING ENZYME GENE ALLOWING LINKAGE ANALYSIS IN FAMILIES WITH GLYCOGEN-STORAGE-DISEASE TYPE-III
Journal of Medical Genetics
COMPARISON OF THE FUNCTIONAL-SIGNIFICANCE OF LEFT-VENTRICULAR HYPERTROPHY IN HYPERTROPHIC CARDIOMYOPATHY AND GLYCOGENOSIS TYPE-III
The American journal of cardiology
EXPRESSION OF CATALYTICALLY ACTIVE HUMAN MULTIFUNCTIONAL GLYCOGEN-DEBRANCHING ENZYME AND LYSOSOMAL ACID ALPHA-GLUCOSIDASE IN INSECT CELLS
Biochemistry and molecular biology international
PURIFICATION AND PROPERTIES OF A 140 KDA AMYLOPULLULANASE FROM THERMOPHILIC AND ALKALIPHILIC BACILLUS SP STRAIN TS-23
Biotechnology and applied biochemistry
SOME PROPERTIES OF STARCH DEBRANCHING ENZYMES AND THEIR POSSIBLE ROLEIN AMYLOPECTIN BIOSYNTHESIS
PLANT SCIENCE
PURIFICATION AND CHARACTERIZATION OF ALPHA-AMYLASE FROM POPLAR LEAVES
Phytochemistry
CHANGES IN STRUCTURE OF STARCH AND ENZYME-ACTIVITIES AFFECTED BY SUGARY MUTATIONS IN DEVELOPING RICE ENDOSPERM - POSSIBLE ROLE OF STARCH DEBRANCHING ENZYME (R-ENZYME) IN AMYLOPECTIN BIOSYNTHESIS
Physiologia Plantarum
MUTATIONS IN EXON-3 OF THE GLYCOGEN DEBRANCHING ENZYME GENE ARE ASSOCIATED WITH GLYCOGEN-STORAGE-DISEASE TYPE-III THAT IS DIFFERENTIALLY EXPRESSED IN LIVER AND MUSCLE
The Journal of clinical investigation
BARLEY MALT LIMIT DEXTRINASE - ITS EXTRACTION, HEAT-STABILITY, AND ACTIVITY DURING MALTING AND MASHING
Journal of the American Society of Brewing Chemists
PURIFICATION AND CHARACTERIZATION OF AN ALKALINE AMYLOPULLULANASE WITH BOTH ALPHA-1,4 AND ALPHA-1,6 HYDROLYTIC ACTIVITY FROM ALKALOPHILIC BACILLUS SP KSM-1378
Biochimica et biophysica acta (G). General subjects
BIOCHEMISTRY AND MOLECULAR-GENETICS OF HUMAN GLYCOGENOSES - AN OVERVIEW
Muscle & nerve
THE HEPATIC GLYCOGEN-STORAGE DISEASES - PROBLEMS BEYOND CHILDHOOD
Journal of inherited metabolic disease
A CASE OF GLYCOGEN-STORAGE-DISEASE TYPE-III (GLYCOGEN DEBRANCHING ENZYME DEFICIENCY) WITH LIVER-CIRRHOSIS AND HYPERTROPHIC CARDIOMYOPATHY
Tohoku Journal of Experimental Medicine
AMYLOLYTIC ACTIVITIES IN CEREAL SEEDS UNDER AEROBIC AND ANAEROBIC CONDITIONS
Plant physiology
LIGHT-INDUCED CHLOROPLAST ALPHA-AMYLASE IN PEARL-MILLET (PENNISETUM-AMERICANUM)
Plant physiology
AN ASSAY FOR THE TRANSFERASE-ACTIVITY OF GLYCOGEN DEBRANCHING ENZYME
Carbohydrate research
EFFECTS OF BAY M-1099, AN ALPHA-GLUCOSIDASE INHIBITOR, ON STARCH METABOLISM IN GERMINATING WHEAT SEEDS
Bioscience, biotechnology, and biochemistry
HYDROLYSIS OF BARLEY STARCH GRANULES BY ALPHA-GLUCOSIDASES FROM MALT
Journal of cereal science
SPECIFIC DETECTION OF PULLULANASE TYPE-I IN POLYACRYLAMIDE GELS
FEMS microbiology letters
ENZYME-ACTIVITIES OF GLYCOGEN-METABOLISM AND MITOCHONDRIAL CHARACTERISTICS IN MUSCLES OF RN(-) CARRIER PIGS (SUS-SCROFA-DOMESTICUS)
Comparative biochemistry and physiology. B. Comparative biochemistry
RANDOM MUTAGENESIS OF PULLULANASE FROM KLEBSIELLA-AEROGENES FOR STUDIES OF THE STRUCTURE AND FUNCTION OF THE ENZYME
Journal of Biochemistry
LIMIT DEXTRINASE FROM MALTED BARLEY - EXTRACTION, PURIFICATION, AND CHARACTERIZATION
Cereal chemistry
GLYCOGEN-STORAGE-DISEASE IN ADULTS
Annals of internal medicine
PURIFICATION AND CHARACTERIZATION OF AN ALKALINE ISOAMYLASE FROM AN ALKALOPHILIC STRAIN OF BACILLUS
Journal of General Microbiology
A MAN WITH TYPE-III GLYCOGENOSIS ASSOCIATED WITH CIRRHOSIS AND PORTAL-HYPERTENSION
Gastroenterology
THE EFFECT OF STORAGE-TEMPERATURE ON REDUCING SUGAR CONCENTRATION ANDTHE ACTIVITIES OF 3 AMYLOLYTIC ENZYMES IN TUBERS OF THE CULTIVATED POTATO, SOLANUM-TUBEROSUM L
Potato research