Identification of isoamylase, a glycogen‐debranching enzyme, from Bacillus amyloliquefaciens [PDF]
FEBS Letters, 1975 Bacillus arnyloliquefaciens is well known for its production of an extracellular cu-amylase. The enzyme is secreted into the culture fluid during the exponential phase of growth when the bacteria are grown with an amylaceous carbon source, e.g. maltose, maltodextrins, soluble starch, or glycogen.
Herbert Urlaub, Günter Wöber
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The Saccharomyces cerevisiae YPR184w gene encodes the glycogen debranching enzyme [PDF]
FEMS Microbiology Letters, 2000 The YPR184w gene encodes a 1536-amino acid protein that is 34-39% identical to the mammal, Drosophila melanogaster and Caenorhabditis elegans glycogen debranching enzyme. The N-terminal part of the protein possesses the four conserved sequences of the alpha-amylase superfamily, while the C-terminal part displays 50% similarity with the C-terminal of ...
Marie-Ange Teste
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Long-Term Correction of Murine Glycogen Storage Disease Type III by AAV-Mediated Gene Therapy Using an Immunotolerizing Dual Promoter to Express Bacterial Pullulanase. [PDF]
Adv Cell Gene TherBackground: We recently reported an innovative gene therapy approach for GSD III using a recombinant adeno‐associated virus serotype 9 vector (AAV9‐Dual‐Pull) expressing a bacterial debranching enzyme (pullulanase) driven by a tandem dual promoter that consists of an immunotolerizing liver‐specific promoter (LSP) and the ubiquitous CMV enhance/chicken ...
Liao KA, Lim JA, Choi SJ, Yi H, Sun B.
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Glycogen debranching enzyme (AGL) is a novel regulator of non-small cell lung cancer growth. [PDF]
Oncotarget, 2018 Richmond CS +6 more
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Laforin-malin complex degrades polyglucosan bodies in concert with glycogen debranching enzyme and brain isoform glycogen phosphorylase. [PDF]
Mol Neurobiol, 2014 In Lafora disease (LD), the deficiency of either EPM2A or NHLRC1, the genes encoding the phosphatase laforin and E3 ligase, respectively, causes massive accumulation of less-branched glycogen inclusions, known as Lafora bodies, also called polyglucosan bodies (PBs), in several types of cells including neurons.
Liu Y +6 more
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Loss of Glycogen Debranching Enzyme AGL Drives Bladder Tumor Growth via Induction of Hyaluronic Acid Synthesis. [PDF]
Clin Cancer Res, 2016 Guin S +6 more
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Glycogen storage disease due to glycogen debranching enzyme deficiency [PDF]
, 2020 INSERM
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High-potency MyoAAV capsids enhanced skeletal muscle correction in a mouse model of GSD IIIa [PDF]
Molecular Therapy: Methods & Clinical DevelopmentGlycogen storage disease type IIIa (GSD IIIa) affects multiple tissues, including liver, heart, and skeletal muscles. We recently reported that an adeno-associated virus serotype 9 vector expressing pullulanase, a bacterial glycogen debranching enzyme ...
Kuo-An Liao +7 more
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Molecular cloning and nucleotide sequence of cDNA encoding human muscle glycogen debranching enzyme.
Journal of Biological Chemistry, 1992 cDNA comprising the entire length of the human muscle glycogen debranching enzyme was cloned and its nucleotide sequence determined. The debrancher mRNA includes a 4545-base pair coding region and a 2371-base pair 3'-nontranslated region. The calculated molecular mass of the debrancher protein derived from cDNA sequence is 172,614 daltons, consistent ...
Bing-Zhi Yang +4 more
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Clinical, biochemical and genetic features of glycogen debranching enzyme deficiency. [PDF]
Acta Myol, 2007 Deficiency of debrancher enzyme causes Glycogen Storage Disease (GSD) type III, an autosomal recessive disorder, characterized by tissue accumulation of abnormally structured glycogen. This report reviews current clinical and molecular knowledge about this disorder and describes the variability at phenotype and genotype levels of a large group of ...
Lucchiari S +3 more
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