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Protein

Glycogen phosphorylase, muscle form

Gene

PYGM

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Phosphorylase is an important allosteric enzyme in carbohydrate metabolism. Enzymes from different sources differ in their regulatory mechanisms and in their natural substrates. However, all known phosphorylases share catalytic and structural properties.

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the ‘Function’ section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

pyridoxal 5'-phosphate

<p>This subsection of the ‘Function’ section describes regulatory mechanisms for enzymes, transporters or microbial transcription factors, and reports the components which regulate (by activation or inhibition) the reaction.<p><a href='/help/activity_regulation' target='_top'>More...</a></p>Activity regulationi

Activity of phosphorylase is controlled both by allosteric means (through the noncovalent binding of metabolites) and by covalent modification. Thus AMP allosterically activates, whereas ATP, ADP, and glucose-6-phosphate allosterically inhibit, phosphorylase B.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Function’ section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei76AMPBy similarity1
<p>This subsection describes interesting single amino acid sites on the sequence that are not defined in any other subsection. This subsection can be displayed in different sections (‘Function’, ‘PTM / Processing’, ‘Pathology and Biotech’) according to its content.<p><a href='/help/site' target='_top'>More...</a></p>Sitei109Involved in the association of subunitsBy similarity1
Sitei143Involved in the association of subunitsBy similarity1
Sitei156May be involved in allosteric controlBy similarity1

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

  • glycogen catabolic process Source: GO_Central
  • glycogen metabolic process Source: ProtInc

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionAllosteric enzyme, Glycosyltransferase, Transferase
Biological processCarbohydrate metabolism, Glycogen metabolism
LigandNucleotide-binding, Pyridoxal phosphate

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

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BioCyci
MetaCyc:HS00949-MONOMER

Reactome - a knowledgebase of biological pathways and processes

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Reactomei
R-HSA-70221 Glycogen breakdown (glycogenolysis)

Protein family/group databases

Carbohydrate-Active enZymes

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CAZyi
GT35 Glycosyltransferase Family 35

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Glycogen phosphorylase, muscle form (EC:2.4.1.1)
Alternative name(s):
Myophosphorylase
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:PYGM
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 11

Organism-specific databases

Eukaryotic Pathogen Database Resources

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EuPathDBi
HostDB:ENSG00000068976.13

Human Gene Nomenclature Database

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HGNCi
HGNC:9726 PYGM

Online Mendelian Inheritance in Man (OMIM)

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MIMi
608455 gene

neXtProt; the human protein knowledge platform

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neXtProti
NX_P11217

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Glycogen storage disease 5 (GSD5)13 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA metabolic disorder resulting in myopathy characterized by exercise intolerance, cramps, muscle weakness and recurrent myoglobinuria.
See also OMIM:232600
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_014002116L → P in GSD5. 2 PublicationsCorresponds to variant dbSNP:rs776680924EnsemblClinVar.1
Natural variantiVAR_014003194R → W in GSD5. 1 PublicationCorresponds to variant dbSNP:rs376581557Ensembl.1
Natural variantiVAR_003431205G → S in GSD5. 5 PublicationsCorresponds to variant dbSNP:rs119103251EnsemblClinVar.1
Natural variantiVAR_014004292L → P in GSD5; rare mutation. 1 PublicationCorresponds to variant dbSNP:rs780375860EnsemblClinVar.1
Natural variantiVAR_014005349E → K in GSD5. 1 Publication1
Natural variantiVAR_003432397L → P in GSD5. 2 PublicationsCorresponds to variant dbSNP:rs1005687078Ensembl.1
Natural variantiVAR_014006488T → N in GSD5. 2 Publications1
Natural variantiVAR_003433543K → T in GSD5. 2 PublicationsCorresponds to variant dbSNP:rs119103252EnsemblClinVar.1
Natural variantiVAR_014007602R → W in GSD5. 1 PublicationCorresponds to variant dbSNP:rs750195683Ensembl.1
Natural variantiVAR_003434655E → K in GSD5. 2 PublicationsCorresponds to variant dbSNP:rs119103253EnsemblClinVar.1
Natural variantiVAR_014008660A → D in GSD5. 2 Publications1
Natural variantiVAR_014009666Q → E in GSD5. 1 PublicationCorresponds to variant dbSNP:rs119103256EnsemblClinVar.1
Natural variantiVAR_014010685N → Y in GSD5. 2 Publications1
Natural variantiVAR_014011686G → R in GSD5. 1 PublicationCorresponds to variant dbSNP:rs144081869EnsemblClinVar.1
Natural variantiVAR_014012687A → P in GSD5. 1 Publication1
Natural variantiVAR_014013704A → V in GSD5. 1 PublicationCorresponds to variant dbSNP:rs1483102315Ensembl.1
Natural variantiVAR_014014709Missing in GSD5; common in Japanese patients. 1 Publication1
Natural variantiVAR_014015798W → R in GSD5. 3 PublicationsCorresponds to variant dbSNP:rs119103258EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Glycogen storage disease

Organism-specific databases

DisGeNET

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DisGeNETi
5837

GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

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GeneReviewsi
PYGM

MalaCards human disease database

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MalaCardsi
PYGM
MIMi232600 phenotype

Open Targets

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OpenTargetsi
ENSG00000068976

Orphanet; a database dedicated to information on rare diseases and orphan drugs

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Orphaneti
368 Glycogen storage disease due to muscle glycogen phosphorylase deficiency

The Pharmacogenetics and Pharmacogenomics Knowledge Base

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PharmGKBi
PA34069

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

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ChEMBLi
CHEMBL3526

Drug and drug target database

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DrugBanki
DB04544 1-Deoxy-1-Acetylamino-Beta-D-Gluco-2-Heptulopyranosonamide
DB04013 1-Deoxy-1-Methoxycarbamido-Beta-D-Gluco-2-Heptulopyranosonamide
DB03657 1-Deoxy-1-Methoxycarbamido-Beta-D-Glucopyranose
DB02320 1-N-Acetyl-Beta-D-Glucosamine
DB02604 2-Deoxy-Glucose-6-Phosphate
DB04044 4-{2-[(3-Nitrobenzoyl)Amino]Phenoxy}Phthalic Acid
DB03383 5-Chloro-1h-Indole-2-Carboxylic Acid [1-(4-Fluorobenzyl)-2-(4-Hydroxypiperidin-1yl)-2-Oxoethyl]Amide
DB02720 Alpha-D-Glucopyranosyl-2-Carboxylic Acid Amide
DB02007 alpha-D-glucose 6-phosphate
DB02843 Alpha-D-Glucose-1-Phosphate
DB01823 Beta-D-Glucopyranose Spirohydantoin
DB02379 Beta-D-Glucose
DB03286 C-(1-Azido-Alpha-D-Glucopyranosyl) Formamide
DB02719 C-(1-Hydrogyl-Beta-D-Glucopyranosyl) Formamide
DB03496 Flavopiridol
DB02348 Fluoro-Phosphite Ion
DB04195 Heptulose-2-Phosphate
DB02519 Indirubin-5-Sulphonate
DB04566 Inosinic Acid
DB04083 N'-Pyridoxyl-Lysine-5'-Monophosphate
DB03218 N-Acetyl-N'-Beta-D-Glucopyranosyl Urea
DB04295 N-Benzoyl-N'-Beta-D-Glucopyranosyl Urea
DB02471 Nojirimycine Tetrazole
DB04522 Phosphonoserine
DB00114 Pyridoxal Phosphate

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

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BioMutai
PYGM

Domain mapping of disease mutations (DMDM)

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DMDMi
3041717

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM / Processing</a> section indicates that the initiator methionine is cleaved from the mature protein.<p><a href='/help/init_met' target='_top'>More...</a></p>Initiator methionineiRemovedBy similarity
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00001885292 – 842Glycogen phosphorylase, muscle formAdd BLAST841

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei2N-acetylserineBy similarity1
Modified residuei15Phosphoserine; by PHK; in form phosphorylase A1 Publication1
Modified residuei204PhosphotyrosineBy similarity1
Modified residuei227PhosphotyrosineBy similarity1
Modified residuei430PhosphoserineBy similarity1
Modified residuei473PhosphotyrosineBy similarity1
Modified residuei514PhosphoserineBy similarity1
Modified residuei681N6-(pyridoxal phosphate)lysineBy similarity1
Modified residuei747PhosphoserineBy similarity1
Modified residuei748PhosphoserineBy similarity1

<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM/processing</a> section describes post-translational modifications (PTMs). This subsection <strong>complements</strong> the information provided at the sequence level or describes modifications for which <strong>position-specific data is not yet available</strong>.<p><a href='/help/post-translational_modification' target='_top'>More...</a></p>Post-translational modificationi

Phosphorylation of Ser-15 converts phosphorylase B (unphosphorylated) to phosphorylase A.1 Publication

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

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EPDi
P11217

jPOST - Japan Proteome Standard Repository/Database

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jPOSTi
P11217

MaxQB - The MaxQuant DataBase

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MaxQBi
P11217

PaxDb, a database of protein abundance averages across all three domains of life

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PaxDbi
P11217

PeptideAtlas

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PeptideAtlasi
P11217

PRoteomics IDEntifications database

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PRIDEi
P11217

ProteomicsDB human proteome resource

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ProteomicsDBi
52721
52722 [P11217-2]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

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iPTMneti
P11217

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

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PhosphoSitePlusi
P11217

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Bgee dataBase for Gene Expression Evolution

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Bgeei
ENSG00000068976 Expressed in 155 organ(s), highest expression level in biceps brachii

CleanEx database of gene expression profiles

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CleanExi
HS_PYGM

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
P11217 HS

Organism-specific databases

Human Protein Atlas

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HPAi
HPA056003

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Homodimer. Dimers associate into a tetramer to form the enzymatically active phosphorylase A.

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

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BioGridi
111795, 15 interactors

Protein interaction database and analysis system

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IntActi
P11217, 25 interactors

STRING: functional protein association networks

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STRINGi
9606.ENSP00000164139

Chemistry databases

BindingDB database of measured binding affinities

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BindingDBi
P11217

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1842
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

Select the link destinations:

Protein Data Bank Europe

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PDBei

Protein Data Bank RCSB

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RCSB PDBi

Protein Data Bank Japan

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PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1Z8DX-ray2.30A1-842[»]

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

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ProteinModelPortali
P11217

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
P11217

Database of comparative protein structure models

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ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

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MobiDBi
Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

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EvolutionaryTracei
P11217

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the glycogen phosphorylase family.Curated

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

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eggNOGi
KOG2099 Eukaryota
COG0058 LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00940000153927

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

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HOGENOMi
HOG000278444

The HOVERGEN Database of Homologous Vertebrate Genes

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HOVERGENi
HBG006848

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
P11217

KEGG Orthology (KO)

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KOi
K00688

Identification of Orthologs from Complete Genome Data

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OMAi
WLEMSIN

Database of Orthologous Groups

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OrthoDBi
240595at2759

Database for complete collections of gene phylogenies

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PhylomeDBi
P11217

TreeFam database of animal gene trees

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TreeFami
TF300309

Family and domain databases

Conserved Domains Database

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CDDi
cd04300 GT1_Glycogen_Phosphorylase, 1 hit

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR011833 Glycg_phsphrylas
IPR000811 Glyco_trans_35
IPR035090 Pyridoxal_P_attach_site

The PANTHER Classification System

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PANTHERi
PTHR11468 PTHR11468, 1 hit

Pfam protein domain database

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Pfami
View protein in Pfam
PF00343 Phosphorylase, 1 hit

PIRSF; a whole-protein classification database

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PIRSFi
PIRSF000460 Pprylas_GlgP, 1 hit

TIGRFAMs; a protein family database

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TIGRFAMsi
TIGR02093 P_ylase, 1 hit

PROSITE; a protein domain and family database

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PROSITEi
View protein in PROSITE
PS00102 PHOSPHORYLASE, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (2)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 <p>This subsection of the ‘Sequence’ section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket
Isoform 1 (identifier: P11217-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MSRPLSDQEK RKQISVRGLA GVENVTELKK NFNRHLHFTL VKDRNVATPR
60 70 80 90 100
DYYFALAHTV RDHLVGRWIR TQQHYYEKDP KRIYYLSLEF YMGRTLQNTM
110 120 130 140 150
VNLALENACD EATYQLGLDM EELEEIEEDA GLGNGGLGRL AACFLDSMAT
160 170 180 190 200
LGLAAYGYGI RYEFGIFNQK ISGGWQMEEA DDWLRYGNPW EKARPEFTLP
210 220 230 240 250
VHFYGHVEHT SQGAKWVDTQ VVLAMPYDTP VPGYRNNVVN TMRLWSAKAP
260 270 280 290 300
NDFNLKDFNV GGYIQAVLDR NLAENISRVL YPNDNFFEGK ELRLKQEYFV
310 320 330 340 350
VAATLQDIIR RFKSSKFGCR DPVRTNFDAF PDKVAIQLND THPSLAIPEL
360 370 380 390 400
MRILVDLERM DWDKAWDVTV RTCAYTNHTV LPEALERWPV HLLETLLPRH
410 420 430 440 450
LQIIYEINQR FLNRVAAAFP GDVDRLRRMS LVEEGAVKRI NMAHLCIAGS
460 470 480 490 500
HAVNGVARIH SEILKKTIFK DFYELEPHKF QNKTNGITPR RWLVLCNPGL
510 520 530 540 550
AEVIAERIGE DFISDLDQLR KLLSFVDDEA FIRDVAKVKQ ENKLKFAAYL
560 570 580 590 600
EREYKVHINP NSLFDIQVKR IHEYKRQLLN CLHVITLYNR IKREPNKFFV
610 620 630 640 650
PRTVMIGGKA APGYHMAKMI IRLVTAIGDV VNHDPAVGDR LRVIFLENYR
660 670 680 690 700
VSLAEKVIPA ADLSEQISTA GTEASGTGNM KFMLNGALTI GTMDGANVEM
710 720 730 740 750
AEEAGEENFF IFGMRVEDVD KLDQRGYNAQ EYYDRIPELR QVIEQLSSGF
760 770 780 790 800
FSPKQPDLFK DIVNMLMHHD RFKVFADYED YIKCQEKVSA LYKNPREWTR
810 820 830 840
MVIRNIATSG KFSSDRTIAQ YAREIWGVEP SRQRLPAPDE AI
Length:842
Mass (Da):97,092
Last modified:January 23, 2007 - v6
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:iEBDB7D80D740B68F
GO
Isoform 2 (identifier: P11217-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     82-169: Missing.

Note: No experimental confirmation available.
Show »
Length:754
Mass (Da):87,317
Checksum:iF5C64CA460DA3FC9
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti791L → W in AAA60231 (PubMed:3447177).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_014002116L → P in GSD5. 2 PublicationsCorresponds to variant dbSNP:rs776680924EnsemblClinVar.1
Natural variantiVAR_014003194R → W in GSD5. 1 PublicationCorresponds to variant dbSNP:rs376581557Ensembl.1
Natural variantiVAR_003431205G → S in GSD5. 5 PublicationsCorresponds to variant dbSNP:rs119103251EnsemblClinVar.1
Natural variantiVAR_014004292L → P in GSD5; rare mutation. 1 PublicationCorresponds to variant dbSNP:rs780375860EnsemblClinVar.1
Natural variantiVAR_014005349E → K in GSD5. 1 Publication1
Natural variantiVAR_003432397L → P in GSD5. 2 PublicationsCorresponds to variant dbSNP:rs1005687078Ensembl.1
Natural variantiVAR_061198414R → G. Corresponds to variant dbSNP:rs11231866EnsemblClinVar.1
Natural variantiVAR_014006488T → N in GSD5. 2 Publications1
Natural variantiVAR_003433543K → T in GSD5. 2 PublicationsCorresponds to variant dbSNP:rs119103252EnsemblClinVar.1
Natural variantiVAR_014007602R → W in GSD5. 1 PublicationCorresponds to variant dbSNP:rs750195683Ensembl.1
Natural variantiVAR_003434655E → K in GSD5. 2 PublicationsCorresponds to variant dbSNP:rs119103253EnsemblClinVar.1
Natural variantiVAR_014008660A → D in GSD5. 2 Publications1
Natural variantiVAR_014009666Q → E in GSD5. 1 PublicationCorresponds to variant dbSNP:rs119103256EnsemblClinVar.1
Natural variantiVAR_014010685N → Y in GSD5. 2 Publications1
Natural variantiVAR_014011686G → R in GSD5. 1 PublicationCorresponds to variant dbSNP:rs144081869EnsemblClinVar.1
Natural variantiVAR_014012687A → P in GSD5. 1 Publication1
Natural variantiVAR_014013704A → V in GSD5. 1 PublicationCorresponds to variant dbSNP:rs1483102315Ensembl.1
Natural variantiVAR_014014709Missing in GSD5; common in Japanese patients. 1 Publication1
Natural variantiVAR_014015798W → R in GSD5. 3 PublicationsCorresponds to variant dbSNP:rs119103258EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting. The information stored in this subsection is used to automatically construct alternative protein sequence(s) for display.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_04304782 – 169Missing in isoform 2. 1 PublicationAdd BLAST88

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

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EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...
DDBJi
Links Updated
M32598
, M32579, M32580, M32581, M32582, M32583, M32584, M32585, M32586, M32587, M32588, M32589, M32590, M32591, M32592, M32593, M32594, M32595, M32596, M32597 Genomic DNA Translation: AAA60231.1
U94777
, U94774, U94775, U94776 Genomic DNA Translation: AAC52081.1
AF066859 mRNA Translation: AAC17451.1
AK056607 mRNA Translation: BAG51762.1
AP001462 Genomic DNA No translation available.
CH471076 Genomic DNA Translation: EAW74284.1
BC126392 mRNA Translation: AAI26393.1
BC130514 mRNA Translation: AAI30515.1
X03031 mRNA Translation: CAA26834.1
M16013 mRNA Translation: AAA36216.1

The Consensus CDS (CCDS) project

More...
CCDSi
CCDS53659.1 [P11217-2]
CCDS8079.1 [P11217-1]

Protein sequence database of the Protein Information Resource

More...
PIRi
A27335

NCBI Reference Sequences

More...
RefSeqi
NP_001158188.1, NM_001164716.1 [P11217-2]
NP_005600.1, NM_005609.3 [P11217-1]

UniGene gene-oriented nucleotide sequence clusters

More...
UniGenei
Hs.154084

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...
Ensembli
ENST00000164139; ENSP00000164139; ENSG00000068976 [P11217-1]
ENST00000377432; ENSP00000366650; ENSG00000068976 [P11217-2]

Database of genes from NCBI RefSeq genomes

More...
GeneIDi
5837

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...
KEGGi
hsa:5837

UCSC genome browser

More...
UCSCi
uc001oax.5 human [P11217-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M32598
, M32579, M32580, M32581, M32582, M32583, M32584, M32585, M32586, M32587, M32588, M32589, M32590, M32591, M32592, M32593, M32594, M32595, M32596, M32597 Genomic DNA Translation: AAA60231.1
U94777
, U94774, U94775, U94776 Genomic DNA Translation: AAC52081.1
AF066859 mRNA Translation: AAC17451.1
AK056607 mRNA Translation: BAG51762.1
AP001462 Genomic DNA No translation available.
CH471076 Genomic DNA Translation: EAW74284.1
BC126392 mRNA Translation: AAI26393.1
BC130514 mRNA Translation: AAI30515.1
X03031 mRNA Translation: CAA26834.1
M16013 mRNA Translation: AAA36216.1
CCDSiCCDS53659.1 [P11217-2]
CCDS8079.1 [P11217-1]
PIRiA27335
RefSeqiNP_001158188.1, NM_001164716.1 [P11217-2]
NP_005600.1, NM_005609.3 [P11217-1]
UniGeneiHs.154084

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1Z8DX-ray2.30A1-842[»]
ProteinModelPortaliP11217
SMRiP11217
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111795, 15 interactors
IntActiP11217, 25 interactors
STRINGi9606.ENSP00000164139

Chemistry databases

BindingDBiP11217
ChEMBLiCHEMBL3526
DrugBankiDB04544 1-Deoxy-1-Acetylamino-Beta-D-Gluco-2-Heptulopyranosonamide
DB04013 1-Deoxy-1-Methoxycarbamido-Beta-D-Gluco-2-Heptulopyranosonamide
DB03657 1-Deoxy-1-Methoxycarbamido-Beta-D-Glucopyranose
DB02320 1-N-Acetyl-Beta-D-Glucosamine
DB02604 2-Deoxy-Glucose-6-Phosphate
DB04044 4-{2-[(3-Nitrobenzoyl)Amino]Phenoxy}Phthalic Acid
DB03383 5-Chloro-1h-Indole-2-Carboxylic Acid [1-(4-Fluorobenzyl)-2-(4-Hydroxypiperidin-1yl)-2-Oxoethyl]Amide
DB02720 Alpha-D-Glucopyranosyl-2-Carboxylic Acid Amide
DB02007 alpha-D-glucose 6-phosphate
DB02843 Alpha-D-Glucose-1-Phosphate
DB01823 Beta-D-Glucopyranose Spirohydantoin
DB02379 Beta-D-Glucose
DB03286 C-(1-Azido-Alpha-D-Glucopyranosyl) Formamide
DB02719 C-(1-Hydrogyl-Beta-D-Glucopyranosyl) Formamide
DB03496 Flavopiridol
DB02348 Fluoro-Phosphite Ion
DB04195 Heptulose-2-Phosphate
DB02519 Indirubin-5-Sulphonate
DB04566 Inosinic Acid
DB04083 N'-Pyridoxyl-Lysine-5'-Monophosphate
DB03218 N-Acetyl-N'-Beta-D-Glucopyranosyl Urea
DB04295 N-Benzoyl-N'-Beta-D-Glucopyranosyl Urea
DB02471 Nojirimycine Tetrazole
DB04522 Phosphonoserine
DB00114 Pyridoxal Phosphate

Protein family/group databases

CAZyiGT35 Glycosyltransferase Family 35

PTM databases

iPTMnetiP11217
PhosphoSitePlusiP11217

Polymorphism and mutation databases

BioMutaiPYGM
DMDMi3041717

Proteomic databases

EPDiP11217
jPOSTiP11217
MaxQBiP11217
PaxDbiP11217
PeptideAtlasiP11217
PRIDEiP11217
ProteomicsDBi52721
52722 [P11217-2]

Protocols and materials databases

The DNASU plasmid repository

More...
DNASUi
5837
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000164139; ENSP00000164139; ENSG00000068976 [P11217-1]
ENST00000377432; ENSP00000366650; ENSG00000068976 [P11217-2]
GeneIDi5837
KEGGihsa:5837
UCSCiuc001oax.5 human [P11217-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
5837
DisGeNETi5837
EuPathDBiHostDB:ENSG00000068976.13

GeneCards: human genes, protein and diseases

More...
GeneCardsi
PYGM
GeneReviewsiPYGM
HGNCiHGNC:9726 PYGM
HPAiHPA056003
MalaCardsiPYGM
MIMi232600 phenotype
608455 gene
neXtProtiNX_P11217
OpenTargetsiENSG00000068976
Orphaneti368 Glycogen storage disease due to muscle glycogen phosphorylase deficiency
PharmGKBiPA34069

GenAtlas: human gene database

More...
GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG2099 Eukaryota
COG0058 LUCA
GeneTreeiENSGT00940000153927
HOGENOMiHOG000278444
HOVERGENiHBG006848
InParanoidiP11217
KOiK00688
OMAiWLEMSIN
OrthoDBi240595at2759
PhylomeDBiP11217
TreeFamiTF300309

Enzyme and pathway databases

BioCyciMetaCyc:HS00949-MONOMER
ReactomeiR-HSA-70221 Glycogen breakdown (glycogenolysis)

Miscellaneous databases

EvolutionaryTraceiP11217

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...
GenomeRNAii
5837

Protein Ontology

More...
PROi
PR:P11217

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000068976 Expressed in 155 organ(s), highest expression level in biceps brachii
CleanExiHS_PYGM
GenevisibleiP11217 HS

Family and domain databases

CDDicd04300 GT1_Glycogen_Phosphorylase, 1 hit
InterProiView protein in InterPro
IPR011833 Glycg_phsphrylas
IPR000811 Glyco_trans_35
IPR035090 Pyridoxal_P_attach_site
PANTHERiPTHR11468 PTHR11468, 1 hit
PfamiView protein in Pfam
PF00343 Phosphorylase, 1 hit
PIRSFiPIRSF000460 Pprylas_GlgP, 1 hit
TIGRFAMsiTIGR02093 P_ylase, 1 hit
PROSITEiView protein in PROSITE
PS00102 PHOSPHORYLASE, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

More...
ProtoNeti
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiPYGM_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P11217
Secondary accession number(s): A0AVK1, A6NDY6
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 1, 1989
Last sequence update: January 23, 2007
Last modified: January 16, 2019
This is version 217 of the entry and version 6 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. Human chromosome 11
    Human chromosome 11: entries, gene names and cross-references to MIM
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