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glycogen phosphorylase L

Summary

Gene Symbol

PYGL

Aliases

GSD6
Hers disease
glycogen phosphorylase, liver form
glycogen storage disease type VI

Organism

Homo sapiens (human)

External Links

NCBI Gene

5836

HGNC

9725

KEGG Gene ID

hsa:5836

PubChem

5836

Alliance of Genome Resources

HGNC:9725

Annotation

Keyword

3D-structure
Acetylation
Allosteric enzyme
Alternative splicing
Cytoplasm
Disease variant
Glycogen metabolism
Glycogen storage disease
Glycosyltransferase
Nucleotide-binding
Phosphoprotein
Pyridoxal phosphate
Reference proteome

Proteins

Displaying 1 entry
UniProt	Protein Name
P06737

Annotation information from UniProt.

Gene Ontology (GO)

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GO Term	Evidence Code	PMID
glycogen metabolic process	IMP	10980448 17705025
glycogen catabolic process	IBA
necroptotic process	IEA
response to bacterium	IEA
glucose homeostasis	IMP	17705025

GO Hierarchy

biological process

metabolic process [inference]

catabolic process [inference]

organic substance catabolic process [inference]

macromolecule catabolic process [inference]

polysaccharide catabolic process [inference]

glucan catabolic process [inference]

glycogen catabolic process

carbohydrate catabolic process [inference]

polysaccharide catabolic process [inference]

glucan catabolic process [inference]

glycogen catabolic process

biosynthetic process [inference]

organic substance biosynthetic process [inference]

organophosphate biosynthetic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

carbohydrate derivative biosynthetic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

cellular metabolic process [inference]

generation of precursor metabolites and energy [inference]

energy derivation by oxidation of organic compounds [inference]

energy reserve metabolic process [inference]

glycogen metabolic process

glycogen catabolic process

phosphorus metabolic process [inference]

phosphate-containing compound metabolic process [inference]

ribose phosphate metabolic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

5-phosphoribose 1-diphosphate metabolic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

organophosphate metabolic process [inference]

ribose phosphate metabolic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

5-phosphoribose 1-diphosphate metabolic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

organophosphate biosynthetic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

primary metabolic process [inference]

carbohydrate metabolic process [inference]

polysaccharide metabolic process [inference]

polysaccharide catabolic process [inference]

glucan catabolic process [inference]

glycogen catabolic process

glucan metabolic process [inference]

glycogen metabolic process

glycogen catabolic process

glucan catabolic process [inference]

glycogen catabolic process

carbohydrate catabolic process [inference]

polysaccharide catabolic process [inference]

glucan catabolic process [inference]

glycogen catabolic process

organic substance metabolic process [inference]

carbohydrate metabolic process [inference]

polysaccharide metabolic process [inference]

polysaccharide catabolic process [inference]

glucan catabolic process [inference]

glycogen catabolic process

glucan metabolic process [inference]

glycogen metabolic process

glycogen catabolic process

glucan catabolic process [inference]

glycogen catabolic process

carbohydrate catabolic process [inference]

polysaccharide catabolic process [inference]

glucan catabolic process [inference]

glycogen catabolic process

organophosphate metabolic process [inference]

ribose phosphate metabolic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

5-phosphoribose 1-diphosphate metabolic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

organophosphate biosynthetic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

macromolecule metabolic process [inference]

polysaccharide metabolic process [inference]

polysaccharide catabolic process [inference]

glucan catabolic process [inference]

glycogen catabolic process

glucan metabolic process [inference]

glycogen metabolic process

glycogen catabolic process

glucan catabolic process [inference]

glycogen catabolic process

macromolecule catabolic process [inference]

polysaccharide catabolic process [inference]

glucan catabolic process [inference]

glycogen catabolic process

carbohydrate derivative metabolic process [inference]

ribose phosphate metabolic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

5-phosphoribose 1-diphosphate metabolic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

carbohydrate derivative biosynthetic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

organic substance catabolic process [inference]

macromolecule catabolic process [inference]

polysaccharide catabolic process [inference]

glucan catabolic process [inference]

glycogen catabolic process

carbohydrate catabolic process [inference]

polysaccharide catabolic process [inference]

glucan catabolic process [inference]

glycogen catabolic process

organic substance biosynthetic process [inference]

organophosphate biosynthetic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

carbohydrate derivative biosynthetic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

cellular process [inference]

cell death [inference]

programmed cell death [inference]

programmed necrotic cell death [inference]

necroptotic process

cellular metabolic process [inference]

generation of precursor metabolites and energy [inference]

energy derivation by oxidation of organic compounds [inference]

energy reserve metabolic process [inference]

glycogen metabolic process

glycogen catabolic process

phosphorus metabolic process [inference]

phosphate-containing compound metabolic process [inference]

ribose phosphate metabolic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

5-phosphoribose 1-diphosphate metabolic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

organophosphate metabolic process [inference]

ribose phosphate metabolic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

5-phosphoribose 1-diphosphate metabolic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

organophosphate biosynthetic process [inference]

ribose phosphate biosynthetic process [inference]

5-phosphoribose 1-diphosphate biosynthetic process

homeostatic process [inference]

chemical homeostasis [inference]

carbohydrate homeostasis [inference]

glucose homeostasis

biological process involved in interspecies interaction between organisms [inference]

response to other organism [inference]

response to bacterium

response to stimulus [inference]

response to external stimulus [inference]

response to external biotic stimulus [inference]

response to other organism [inference]

response to bacterium

response to biotic stimulus [inference]

response to external biotic stimulus [inference]

response to other organism [inference]

response to bacterium

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GO Term	Evidence Code	PMID
cytosol	TAS
extracellular region	TAS
cytoplasm	IBA
secretory granule lumen	TAS
ficolin-1-rich granule lumen	TAS

GO Hierarchy

cellular component

cellular anatomical entity [inference]

membrane-enclosed lumen [inference]

organelle lumen [inference]

vesicle lumen [inference]

cytoplasmic vesicle lumen [inference]

secretory granule lumen

intracellular organelle lumen [inference]

cytoplasmic vesicle lumen [inference]

secretory granule lumen

ficolin-1-rich granule lumen

organelle [inference]

membrane-bounded organelle [inference]

vesicle [inference]

extracellular vesicle [inference]

extracellular exosome

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

extracellular organelle [inference]

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

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GO Term	Evidence Code	PMID
linear malto-oligosaccharide phosphorylase activity	IEA
glucose binding	IDA	10980448 12204691
SHG alpha-glucan phosphorylase activity	IEA
bile acid binding	IDA	12204691
purine nucleobase binding	IDA	12204691

GO Hierarchy

molecular function

catalytic activity [inference]

transferase activity [inference]

glycosyltransferase activity [inference]

hexosyltransferase activity [inference]

1,4-alpha-oligoglucan phosphorylase activity [inference]

glycogen phosphorylase activity

binding [inference]

protein binding

identical protein binding

carbohydrate binding [inference]

monosaccharide binding [inference]

glucose binding

small molecule binding [inference]

vitamin binding

vitamin B6 binding [inference]

pyridoxal phosphate binding

organic acid binding [inference]

carboxylic acid binding [inference]

monocarboxylic acid binding [inference]

bile acid binding

monosaccharide binding [inference]

glucose binding

heterocyclic compound binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

nucleobase binding [inference]

purine nucleobase binding

vitamin B6 binding [inference]

pyridoxal phosphate binding

ion binding [inference]

anion binding [inference]

carboxylic acid binding [inference]

monocarboxylic acid binding [inference]

bile acid binding

purine ribonucleoside triphosphate binding [inference]

ATP binding

cation binding [inference]

AMP binding

organic cyclic compound binding [inference]

nucleobase binding [inference]

purine nucleobase binding

vitamin B6 binding [inference]

pyridoxal phosphate binding

nucleoside phosphate binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

purine ribonucleoside triphosphate binding [inference]

ATP binding

carbohydrate derivative binding [inference]

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

Gene Ontology information from NCBI Gene and UniProt.

Human Protein Atlas

ENSG00000100504

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

KEGG BRITE Database

Orthology

K00688

Name

glycogen phosphorylase [EC:2.4.1.1]

References

2877458

Rhea

RHEA:41732

phosphate

α-D-glucose 1-phosphate

Enzyme

2.4.1.1

PMID

Disease

Disease Ontology

Displaying entries **1 - 10** of 18 in total

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DO ID	Disease Name	Source
DOID:11721	glycogen storage disease VII	DisGeNET
DOID:2746	glycogen storage disease V	DisGeNET
DOID:2747	glycogen storage disease	DisGeNET Alliance of Genome Resources
DOID:2748	glycogen storage disease III	DisGeNET
DOID:2749	glycogen storage disease Ia	DisGeNET
DOID:2750	glycogen storage disease IV	DisGeNET
DOID:2754	glycogen storage disease VI	DisGeNET Alliance of Genome Resources
DOID:2862	glucosephosphate dehydrogenase deficiency	DisGeNET
DOID:3571	liver cancer	DisGeNET
DOID:3650	lactic acidosis	Alliance of Genome Resources

The Human Phenotype Ontology

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HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000077	Abnormality of the kidney
HP:0000093	Proteinuria
HP:0000737	Irritability
HP:0000823	Delayed puberty
HP:0000938	Osteopenia
HP:0000939	Osteoporosis
HP:0001252	Hypotonia
HP:0001270	Motor delay
HP:0001394	Cirrhosis

Displaying **all 2** entries
Disease ID	Disease Name
ORPHA:369	glycogen storage disease VI
OMIM:232700	glycogen storage disease VI

PubChem Disease

GHR Health Conditions

Glycogen storage disease type VI

MedGen Diseases

Glycogen storage disease, type VI

OMIM Phenotypes

GLYCOGEN STORAGE DISEASE VI; GSD6

KEGG Diseases

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

Ortholog

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Caenorhabditis elegans	178777	WB:WBGene00020696
Drosophila melanogaster	33386	FB:FBgn0004507
Danio rerio	493916	ZFIN:ZDB-GENE-041205-1
Astyanax mexicanus	103026524		ASTMX05807
Electrophorus electricus	113583453		ELEEL31516
Salmo salar	106604778		SALSA43538
Salmo salar	106611459		SALSA43538
Salmo trutta	115161899		SALTR57493
Salmo trutta	115172973		SALTR72144
Oreochromis niloticus	100694493		ORENI32645

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

All Resources

Other Information

Tools

Search Tools

Summary

External Links

Annotation

KEGG BRITE Database

PubChem Disease

International Collaboration

Acknowledgements