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Details on Person UniProt:P46976 GYG1

Class:IdReferenceGeneProduct:55746
_chainChangeLoginitiator methionine:1 added on Fri February 6 2015;chain:2-350 added on Fri February 6 2015;initiator methionine:1 for 55746 removed on Fri Nov 03 2023;initiator methionine: for 55746 added on Fri Nov 03 2023;initiator methionine: for 55746 removed on Fri Aug 15 2025;initiator methionine:1 for 55746 added on Fri Aug 15 2025
_displayNameUniProt:P46976 GYG1
_timestamp2025-08-15 21:22:52
chaininitiator methionine:1
chain:2-350
checksumABAEEB7160DEC4DF
commentFUNCTION Glycogenin participates in the glycogen biosynthetic process along with glycogen synthase and glycogen branching enzyme. It catalyzes the formation of a short alpha (1,4)-glucosyl chain covalently attached via a glucose 1-O-tyrosyl linkage to internal tyrosine residues and these chains act as primers for the elongation reaction catalyzed by glycogen synthase.CATALYTIC ACTIVITY L-tyrosyl-[glycogenin] + UDP-alpha-D-glucose = alpha-D-glucosyl-L-tyrosyl-[glycogenin] + UDP + H(+)CATALYTIC ACTIVITY [1,4-alpha-D-glucosyl](n)-L-tyrosyl-[glycogenin] + UDP-alpha-D-glucose = [1,4-alpha-D-glucosyl](n+1)-L-tyrosyl-[glycogenin] + UDP + H(+)COFACTOR Divalent metal ions. Required for self-glucosylation. Manganese is the most effective.ACTIVITY REGULATION Inhibited by palladium ions.PATHWAY Glycan biosynthesis; glycogen biosynthesis.SUBUNIT Part of the GYS1-GYG1 complex, a heterooctamer composed of a tetramer of GYS1 and 2 dimers of GYG1, where each GYS1 protomer binds to one GYG1 subunit (via GYG1 C-terminus); the GYS1 tetramer may dissociate from GYG1 dimers to continue glycogen polymerization on its own (PubMed:17055998, PubMed:22160680, PubMed:35690592, PubMed:35835870). May also form a heterooctamer complex with GYS2 (via GYG1 C-terminus) (By similarity).INTERACTION Localizes to glycogen granules (glycosomes) in the cytoplasm (By similarity). Cytosolic localization is dependent on the actin cytoskeleton (By similarity).ALTERNATIVE PRODUCTS Highly expressed in skeletal muscle and heart, with lower levels in brain, lung, kidney and pancreas.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.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the glycosyltransferase 8 family. Glycogenin subfamily.
descriptionrecommendedName: fullName evidence="17"Glycogenin-1 shortName: GN-1 shortName: GN1 ecNumber evidence="6 8"2.4.1.186
geneNameGYG1
GYG
identifierP46976
isSequenceChangedFALSE
keyword3D-structure
Acetylation
Alternative splicing
Cytoplasm
Disease variant
Glycogen biosynthesis
Glycogen storage disease
Glycoprotein
Manganese
Metal-binding
Nucleus
Phosphoprotein
Proteomics identification
Reference proteome
Transferase
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9862192] Weiser, Joel, 2024-02-26
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
nameGYG1
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8991290] ENSEMBL:ENSG00000163754 GYG1 [Homo sapiens]
secondaryIdentifierGLYG_HUMAN
D3DNH0
D3DNH1
D3DNH2
Q6FHZ1
Q9UNV0
sequenceLength350
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:148068] UniProt:P46976-2 GYG1 [Homo sapiens]
[ReferenceIsoform:148069] UniProt:P46976-3 GYG1 [Homo sapiens]
[ReferenceIsoform:404105] UniProt:P46976-1 GYG1 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:70174] glycogen-GYG1 [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:70179] limit dextrin-glycogenin-1 [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:70186] poly((1,4)-alpha-glucosyl)GYG1 [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:70190] GYG1 [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:70292] oligo((1,4)-alpha-glucosyl) GYG1 [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:70298] ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)GYG1 [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:3777092] polysaccharide-P-GYG1 [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:3814832] GYG1 T83M [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:5357542] ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)GYG1 [lysosomal lumen] [Homo sapiens]
[EntityWithAccessionedSequence:5357545] poly((1,4)-alpha-glucosyl)GYG1 [lysosomal lumen] [Homo sapiens]
List all 16 refering instances
(referenceSequence)[GroupModifiedResidue:70173] O4'-glucosyl-L-tyrosine (glycogen group) at 195
[GroupModifiedResidue:70178] O4'-glucosyl-L-tyrosine ((1,4-alpha-D-glucosyl)(n)-L-tyrosyl residue) at 194
[GroupModifiedResidue:70185] O4'-glucosyl-L-tyrosine ((1,4-alpha-D-glucosyl)(n)-L-tyrosyl residue) at 195
[GroupModifiedResidue:70291] O4'-glucosyl-L-tyrosine (alpha-D-glucosyl {alpha-D-glucosyl-(1->4)}n-alpha-D-glucopyranoside) at 195
[GroupModifiedResidue:70297] O4'-glucosyl-L-tyrosine (glycogen (amylose chain)n-[(1->6)-amylose chain]4) at 194
[GroupModifiedResidue:3777098] O4'-glucosyl-L-tyrosine (polysaccharide phosphate group) at 195
[ReplacedResidue:3814834] L-threonine 83 replaced with L-methionine
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No pathways have been reviewed or authored by UniProt:P46976 GYG1 (55746)