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Glycogenin 1

Glycogenin, GYG, glycogenin-1, GLG1
This gene encodes a member of the glycogenin family. Glycogenin is a glycosyltransferase that catalyzes the formation of a short glucose polymer from uridine diphosphate glucose in an autoglucosylation reaction. This reaction is followed by elongation and branching of the polymer, catalyzed by glycogen synthase and branching enzyme, to form glycogen. This gene is expressed in muscle and other tissues. Mutations in this gene result in glycogen storage disease XV. This gene has pseudogenes on chromosomes 1, 8 and 13 respectively. Alternatively spliced transcript variants encoding different isoforms have been identified.[provided by RefSeq, Sep 2010] (from NCBI)
Top mentioned proteins: ACID, CAN, fibrillin-1, HAD, alpha-amylase
Papers on Glycogenin
Hypoglycaemic effects of functional tri-peptides from silk in differentiated adipocytes and streptozotocin-induced diabetic mice.
New
Park et al., South Korea. In J Sci Food Agric, Feb 2016
BACKGROUND: In this study, the tri-peptides Gly-Glu-Tyr (GEY) and Gly-Tyr-Gly (GYG), identified previously as active compounds from the silk peptide E5K6, significantly stimulated basal and insulin-mediated glucose uptake by 3T3-L1 fibroblasts in a dose-dependent manner.
Late-onset polyglucosan body myopathy in five patients with a homozygous mutation in GYG1.
New
Marrosu et al., New York City, United States. In Neuromuscul Disord, Dec 2015
The recent description of late-onset polyglucosan myopathy has prompted us to find new genetic defects in the gene (GYG1) encoding glycogenin-1, the crucial primer enzyme of glycogen synthesis in muscle.
Muscle pathology and whole-body MRI in a polyglucosan myopathy associated with a novel glycogenin-1 mutation.
New
Zhao et al., Shanghai, China. In Neuromuscul Disord, Oct 2015
Genetic analysis revealed a homozygous novel mutation in exon 6 of the glycogenin-1 gene (GYG1) (c.634C>T, p.His212Tyr).
High diagnostic yield of clinical exome sequencing in Middle Eastern patients with Mendelian disorders.
New
Ben-Omran et al., Doha, Qatar. In Hum Genet, Sep 2015
In 5% (7/149) of cases, CES implicated novel candidate disease genes (MANF, GJA9, GLG1, COL15A1, SLC35F5, MAGE4, NEUROG1).
TMEM175 Is an Organelle K(+) Channel Regulating Lysosomal Function.
New
Impact
Ren et al., Philadelphia, United States. In Cell, Sep 2015
Unlike any of the ∼80 plasma membrane K(+) channels, TMEM175 has two repeats of 6-transmembrane-spanning segments and has no GYG K(+) channel sequence signature-containing, pore-forming P loop.
Rat skeletal muscle glycogen degradation pathways reveal differential association of glycogen-related proteins with glycogen granules.
New
Murphy et al., Melbourne, Australia. In J Physiol Biochem, Jun 2015
Glycogenin, glycogen-debranching enzyme (GDE) and glycogen phosphorylase (GP) are important enzymes that contribute to glycogen particle metabolism.
Glycogenin-2 is dispensable for liver glycogen synthesis and glucagon-stimulated glucose release.
New
Njølstad et al., Bergen, Norway. In J Clin Endocrinol Metab, May 2015
In humans, glycogenin-1 is expressed ubiquitously, whereas glycogenin-2 (GN2) is highly expressed in liver.
Expression and purification of functional human glycogen synthase-1:glycogenin-1 complex in insect cells.
New
Sakamoto et al., Lausanne, Switzerland. In Protein Expr Purif, Apr 2015
We report the successful expression and purification of functional human muscle glycogen synthase (GYS1) in complex with human glycogenin-1 (GN1).
A new muscle glycogen storage disease associated with glycogenin-1 deficiency.
Oldfors et al., Göteborg, Sweden. In Ann Neurol, 2014
Genetic investigation revealed homozygous or compound heterozygous deleterious variants in the glycogenin-1 gene (GYG1).
LC-MS/MS characterization of combined glycogenin-1 and glycogenin-2 enzymatic activities reveals their self-glucosylation preferences.
Nilsson et al., Göteborg, Sweden. In Biochim Biophys Acta, 2014
Glycogen synthesis is initiated by self-glucosylation of the glycosyltransferases glycogenin-1 and -2 that, in the presence of UDP-glucose, form both the first glucose-O-tyrosine linkage, and then stepwise add a series of α1,4-linked glucoses to a growing chain of variable length.
Comparative proteomics analysis of gastric cancer stem cells.
Hirakawa et al., Ōsaka, Japan. In Plos One, 2013
Based on the results of LC-MS/MS, eight proteins, including RBBP6, GLG1, VPS13A, DCTPP1, HSPA9, HSPA4, ALDOA, and KRT18, were up-regulated in both OCUM-12/SP cells and OCUM-2MD3/SP cells when compared to their corresponding parent cells.
Effect of muscle-fiber type on glycogenin-1 gene expression and its relationship with the glycolytic potential and pH of pork.
Li et al., China. In Genet Mol Res, 2012
This study analyzed the effect of muscle-fiber type composition on glycogenin-1 (GYG) gene expression and its impact on pH.
Molecular pathogenesis of a new glycogenosis caused by a glycogenin-1 mutation.
GeneRIF
Oldfors et al., Göteborg, Sweden. In Biochim Biophys Acta, 2012
results explain the glycogen depletion in the patient expressing only Thr83Met glycogenin-1 and why heterozygous carriers without clinical symptoms show a small proportion of unglucosylated glycogenin-1
Glycogenin-1 deficiency and inactivated priming of glycogen synthesis.
Impact
Oldfors et al., Göteborg, Sweden. In N Engl J Med, 2010
Sequencing of the glycogenin-1 gene, GYG1, revealed a nonsense mutation in one allele and a missense mutation, Thr83Met, in the other.
Modulation of glycogen synthesis by RNA interference: towards a new therapeutic approach for glycogenosis type II.
GeneRIF
Richard et al., Paris, France. In Hum Mol Genet, 2009
Injection of recombinant adeno-associated virus-1 vector expressing glycogen synthase short hairpin ribonucleic acids into mice with type II glycogen storage disease leads to significant reduction in impaired glycogen accumulation.
Glycogenin protein and mRNA expression in response to changing glycogen concentration in exercise and recovery.
GeneRIF
Graham et al., Guelph, Canada. In Am J Physiol Endocrinol Metab, 2007
GN-1 is conserved, possibly as very small, or nascent, granules when glycogen concentration is low. This would provide the ability to rapidly restore glycogen during early recovery.
Increases in glycogenin and glycogenin mRNA accompany glycogen resynthesis in human skeletal muscle.
GeneRIF
Graham et al., Guelph, Canada. In Am J Physiol Endocrinol Metab, 2005
during recovery from prolonged exhaustive exercise, glycogenin mRNA and protein content and activity increase in muscle
Glycogen synthesis in the absence of glycogenin in the yeast Saccharomyces cerevisiae.
GeneRIF
Parrou et al., Toulouse, France. In Febs Lett, 2005
glycogen synthesis in the absence of glycogenin may rely on an activated glycogen synthase and alternative primers whose synthesis may be controlled by TPS1 or under epigenetic silencing
Glycogenin: the primer for mammalian and yeast glycogen synthesis.
Review
Whelan et al., Miami, United States. In Biochim Biophys Acta, 2004
Initiation in muscle, by a self-glucosylating protein, glycogenin-1, is the most thoroughly studied system, as is described here.
A new look at the biogenesis of glycogen.
Review
Whelan et al., Miami, United States. In Faseb J, 1995
The discovery of glycogenin as a self-glucosylating protein that primes glycogen synthesis has significantly increased our understanding of the structure and metabolism of this storage polysaccharide.
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