Glycogenin is Dispensable for Glycogen Synthesis in Human Muscle, and Glycogenin Deficiency Causes Polyglucosan Storage [PDF]
Glycogenin is considered to be an essential primer for glycogen biosynthesis. Nevertheless, patients with glycogenin-1 deficiency due to biallelic GYG1 (NM_004130.3) mutations can store glycogen in muscle.
Dellgren, Göran+10 more
core +1 more source
Discovery and development of small-molecule inhibitors of glycogen synthase.
The over-accumulation of glycogen appears as a hallmark in various glycogen storage diseases (GSDs), including Pompe, Cori, Andersen and Lafora disease.
B. Tang+12 more
semanticscholar +1 more source
Glycogen and its metabolism: some new developments and old themes [PDF]
Glycogen is a branched polymer of glucose that acts as a store of energy in times of nutritional sufficiency for utilization in times of need. Its metabolism has been the subject of extensive investigation and much is known about its regulation by ...
Depaoli-Roach, Anna A.+3 more
core +1 more source
Allosteric Regulation of Glycogen Synthase Controls Glycogen Synthesis in Muscle [PDF]
Glycogen synthase (GS), a key enzyme in glycogen synthesis, is activated by the allosteric stimulator glucose-6-phosphate (G6P) and by dephosphorylation through inactivation of GS kinase-3 with insulin. The relative importance of these two regulatory mechanisms in controlling GS is not established, mainly due to the complex interplay between multiple ...
Kei Sakamoto+9 more
openaire +5 more sources
Cereal and nonfat milk support muscle recovery following exercise [PDF]
All authors are with the Exercise Physiology and Metabolism Laboratory Department of Kinesiology and Health Education The University of Texas at Austin Austin, TX, USABackground: This study compared the effects of ingesting cereal and nonfat milk (Cereal)
Ding, Zhenping+5 more
core +3 more sources
Crystal Structure of an Archaeal Glycogen Synthase [PDF]
Glycogen and starch synthases are retaining glycosyltransferases that catalyze the transfer of glucosyl residues to the non-reducing end of a growing alpha-1,4-glucan chain, a central process of the carbon/energy metabolism present in almost all living organisms.
Horcajada, Cristina+3 more
openaire +4 more sources
Rag GTPases are cardioprotective by regulating lysosomal function. [PDF]
The Rag family proteins are Ras-like small GTPases that have a critical role in amino-acid-stimulated mTORC1 activation by recruiting mTORC1 to lysosome.
Guan, Kun-Liang+8 more
core +5 more sources
Nuclear glycogen synthase — fact or artifact? [PDF]
According to Oron [FEBS Lett. (1980) 118, 255–258], nuclear glycogen synthase represents an artifact of preparation in rat liver nuclei. We investigated the nuclei isolated from in vitro growing HD33 ascites cells with exclusively cytoplasmic, and from in vivo growing HD33 Ehrlich—Lettré ascites tumor cells with mainly intranuclear, glycogen deposition.
Christof Granzow+2 more
openaire +3 more sources
Separation and Characterisation of Glycogen Synthase Kinase 3,Glycogen Synthase Kinase 4 and Glycogen Synthase Kinase 5 from Rabbit Skeletal Muscle [PDF]
Three glycogen synthase kinases termed glycogen synthase kinase 3, glycogen synthase kinase 4 and glycogen synthase kinase 5, which are distinct from cyclic‐AMP‐dependent protein kinase and phosphorylase kinase,have been purified and characterised. Their activities are unaffected by Ca2+, calmodulin, cyclic nucleotides and the inhibitor protein of ...
Cohen P+5 more
openaire +3 more sources
Lafora disease offers a unique window into neuronal glycogen metabolism [PDF]
Lafora disease (LD) is a fatal, autosomal recessive, glycogen-storage disorder that manifests as severe epilepsy. LD results from mutations in the gene encoding either the glycogen phosphatase laforin or the E3 ubiquitin ligase malin. Individuals with LD
Gentry, Matthew S.+4 more
core +2 more sources