ID:GLYG_HUMAN DESCRIPTION: RecName: Full=Glycogenin-1; Short=GN-1; Short=GN1; EC=2.4.1.186; FUNCTION: Self-glucosylates, via an inter-subunit mechanism, to form an oligosaccharide primer that serves as substrate for glycogen synthase. CATALYTIC ACTIVITY: UDP-alpha-D-glucose + glycogenin = UDP + alpha-D-glucosylglycogenin. COFACTOR: Divalent metal ions. Required for self-glucosylation. Manganese is the most effective. PATHWAY: Glycan biosynthesis; glycogen biosynthesis. SUBUNIT: Homodimer tightly complexed to the 86 kDa catalytic subunit of glycogen synthase GYS1. INTERACTION: P13807:GYS1; NbExp=4; IntAct=EBI-740533, EBI-740553; PTM: Self-glycosylated by the transfer of glucose residues from UDP-glucose to itself, forming an alpha-1,4-glycan of around 10 residues attached to Tyr-195. PTM: Phosphorylated (By similarity). DISEASE: Defects in GYG1 are the cause of glycogen storage disease type 15 (GSD15) [MIM:613507]. It is a metabolic disorder resulting in muscle weakness, associated with the glycogen depletion in skeletal muscle, and cardiac arrhythmia, associated with the accumulation of abnormal storage material in the heart. The skeletal muscle shows a marked predominance of slow-twitch, oxidative muscle fibers and mitochondrial proliferation. SIMILARITY: Belongs to the glycosyltransferase 8 family. Glycogenin subfamily. SEQUENCE CAUTION: Sequence=CAA56073.1; Type=Frameshift; Positions=204;
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on P46976
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
