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Protein

Glycogen phosphorylase, liver form

Gene

PYGL

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

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.

Catalytic activityi

((1->4)-alpha-D-glucosyl)(n) + phosphate = ((1->4)-alpha-D-glucosyl)(n-1) + alpha-D-glucose 1-phosphate.

Cofactori

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
Binding sitei76AMPBy similarity1
Sitei109Involved in the association of subunitsBy similarity1
Sitei143Involved in the association of subunitsBy similarity1
Sitei156May be involved in allosteric controlBy similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

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

Enzyme and pathway databases

BioCyciMetaCyc:HS02099-MONOMER
BRENDAi2.4.1.1 2681
ReactomeiR-HSA-6798695 Neutrophil degranulation
R-HSA-70221 Glycogen breakdown (glycogenolysis)
SIGNORiP06737

Protein family/group databases

CAZyiGT35 Glycosyltransferase Family 35

Names & Taxonomyi

Protein namesi
Recommended name:
Glycogen phosphorylase, liver form (EC:2.4.1.1)
Gene namesi
Name:PYGL
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 14

Organism-specific databases

EuPathDBiHostDB:ENSG00000100504.16
HGNCiHGNC:9725 PYGL
MIMi613741 gene
neXtProtiNX_P06737

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

Pathology & Biotechi

Involvement in diseasei

Glycogen storage disease 6 (GSD6)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected.
See also OMIM:232700
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_007908339N → S in GSD6. 1 PublicationCorresponds to variant dbSNP:rs113993976EnsemblClinVar.1
Natural variantiVAR_007909377N → K in GSD6. 1 PublicationCorresponds to variant dbSNP:rs113993977EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Glycogen storage disease

Organism-specific databases

DisGeNETi5836
MalaCardsiPYGL
MIMi232700 phenotype
OpenTargetsiENSG00000100504
Orphaneti369 Glycogen storage disease due to liver glycogen phosphorylase deficiency
PharmGKBiPA34068

Chemistry databases

ChEMBLiCHEMBL2568
DrugBankiDB02320 1-N-Acetyl-Beta-D-Glucosamine
DB03288 5-Chloro-1h-Indole-2-Carboxylic Acid{[Cyclopentyl-(2-Hydroxy-Ethyl)-Carbamoyl]-Methyl}-Amide
DB00131 Adenosine monophosphate
DB02379 Beta-D-Glucose
DB02089 CP-526423
DB07968 N-(2-CHLORO-4-FLUOROBENZOYL)-N'-(5-HYDROXY-2-METHOXYPHENYL)UREA
DB04522 Phosphonoserine
DB05044 PSN357
DB00114 Pyridoxal Phosphate
DB08844 Uric Acid

Polymorphism and mutation databases

BioMutaiPYGL
DMDMi6648082

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources
ChainiPRO_00001885242 – 847Glycogen phosphorylase, liver formAdd BLAST846

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylalanineCombined sources1
Modified residuei15Phosphoserine; by PHK; in form phosphorylase A1 Publication1
Modified residuei364N6-succinyllysineBy similarity1
Modified residuei524PhosphoserineBy similarity1
Modified residuei561PhosphoserineBy similarity1
Modified residuei639PhosphoserineCombined sources1
Modified residuei681N6-(pyridoxal phosphate)lysineBy similarity1

Post-translational modificationi

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

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP06737
MaxQBiP06737
PaxDbiP06737
PeptideAtlasiP06737
PRIDEiP06737
ProteomicsDBi51923

PTM databases

iPTMnetiP06737
PhosphoSitePlusiP06737

Expressioni

Gene expression databases

BgeeiENSG00000100504 Expressed in 200 organ(s), highest expression level in blood
CleanExiHS_PYGL
ExpressionAtlasiP06737 baseline and differential
GenevisibleiP06737 HS

Organism-specific databases

HPAiHPA000962
HPA004119

Interactioni

Subunit structurei

Homodimer. Dimers associate into a tetramer to form the enzymatically active phosphorylase A. Interacts with PPP1R3B (By similarity).By similarity

Binary interactionsi

WithEntry#Exp.IntActNotes
PYGBP112168EBI-2511865,EBI-1047231

GO - Molecular functioni

Protein-protein interaction databases

BioGridi111794, 50 interactors
IntActiP06737, 15 interactors
MINTiP06737
STRINGi9606.ENSP00000216392

Chemistry databases

BindingDBiP06737

Structurei

Secondary structure

1847
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP06737
SMRiP06737
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP06737

Family & Domainsi

Sequence similaritiesi

Belongs to the glycogen phosphorylase family.Curated

Phylogenomic databases

eggNOGiKOG2099 Eukaryota
COG0058 LUCA
GeneTreeiENSGT00390000016886
HOVERGENiHBG006848
InParanoidiP06737
KOiK00688
OMAiYPNDNTP
OrthoDBiEOG091G03RB
PhylomeDBiP06737
TreeFamiTF300309

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

Sequences (2+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All

Isoform 1 (identifier: P06737-1) [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
MAKPLTDQEK RRQISIRGIV GVENVAELKK SFNRHLHFTL VKDRNVATTR
60 70 80 90 100
DYYFALAHTV RDHLVGRWIR TQQHYYDKCP KRVYYLSLEF YMGRTLQNTM
110 120 130 140 150
INLGLQNACD EAIYQLGLDI EELEEIEEDA GLGNGGLGRL AACFLDSMAT
160 170 180 190 200
LGLAAYGYGI RYEYGIFNQK IRDGWQVEEA DDWLRYGNPW EKSRPEFMLP
210 220 230 240 250
VHFYGKVEHT NTGTKWIDTQ VVLALPYDTP VPGYMNNTVN TMRLWSARAP
260 270 280 290 300
NDFNLRDFNV GDYIQAVLDR NLAENISRVL YPNDNFFEGK ELRLKQEYFV
310 320 330 340 350
VAATLQDIIR RFKASKFGST RGAGTVFDAF PDQVAIQLND THPALAIPEL
360 370 380 390 400
MRIFVDIEKL PWSKAWELTQ KTFAYTNHTV LPEALERWPV DLVEKLLPRH
410 420 430 440 450
LEIIYEINQK HLDRIVALFP KDVDRLRRMS LIEEEGSKRI NMAHLCIVGS
460 470 480 490 500
HAVNGVAKIH SDIVKTKVFK DFSELEPDKF QNKTNGITPR RWLLLCNPGL
510 520 530 540 550
AELIAEKIGE DYVKDLSQLT KLHSFLGDDV FLRELAKVKQ ENKLKFSQFL
560 570 580 590 600
ETEYKVKINP SSMFDVQVKR IHEYKRQLLN CLHVITMYNR IKKDPKKLFV
610 620 630 640 650
PRTVIIGGKA APGYHMAKMI IKLITSVADV VNNDPMVGSK LKVIFLENYR
660 670 680 690 700
VSLAEKVIPA TDLSEQISTA GTEASGTGNM KFMLNGALTI GTMDGANVEM
710 720 730 740 750
AEEAGEENLF IFGMRIDDVA ALDKKGYEAK EYYEALPELK LVIDQIDNGF
760 770 780 790 800
FSPKQPDLFK DIINMLFYHD RFKVFADYEA YVKCQDKVSQ LYMNPKAWNT
810 820 830 840
MVLKNIAASG KFSSDRTIKE YAQNIWNVEP SDLKISLSNE SNKVNGN
Length:847
Mass (Da):97,149
Last modified:January 23, 2007 - v4
Checksum:i74017E8125FB5735
GO
Isoform 2 (identifier: P06737-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     82-115: Missing.

Note: No experimental confirmation available.
Show »
Length:813
Mass (Da):93,134
Checksum:i40B7DB0528A64AAA
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
E9PK47E9PK47_HUMAN
Alpha-1,4 glucan phosphorylase
PYGL
819Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti2 – 3AK → GE in AAA52577 (PubMed:2877458).Curated2
Sequence conflicti2 – 3AK → GE in AAC18079 (PubMed:9536091).Curated2
Sequence conflicti83V → E in AAA52577 (PubMed:2877458).Curated1
Sequence conflicti323A → Q in AAA52577 (PubMed:2877458).Curated1
Sequence conflicti344 – 345AL → RI in AAA52577 (PubMed:2877458).Curated2
Sequence conflicti369T → N in AAA52577 (PubMed:2877458).Curated1
Sequence conflicti369T → N in AAC18079 (PubMed:9536091).Curated1
Sequence conflicti570R → S in AAC17450 (Ref. 2) Curated1
Sequence conflicti683Missing in AAH09895 (PubMed:15489334).Curated1
Sequence conflicti715R → G in BAG62279 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_007907222V → I1 PublicationCorresponds to variant dbSNP:rs946616EnsemblClinVar.1
Natural variantiVAR_013095231V → E. Corresponds to variant dbSNP:rs1042195Ensembl.1
Natural variantiVAR_007908339N → S in GSD6. 1 PublicationCorresponds to variant dbSNP:rs113993976EnsemblClinVar.1
Natural variantiVAR_007909377N → K in GSD6. 1 PublicationCorresponds to variant dbSNP:rs113993977EnsemblClinVar.1
Natural variantiVAR_034425425R → P1 PublicationCorresponds to variant dbSNP:rs2228499Ensembl.1
Natural variantiVAR_034426698V → G. Corresponds to variant dbSNP:rs35831273Ensembl.1
Natural variantiVAR_013096715R → S. Corresponds to variant dbSNP:rs1042210Ensembl.1
Natural variantiVAR_034427806I → L. Corresponds to variant dbSNP:rs34313873EnsemblClinVar.1
Natural variantiVAR_069054845N → S1 PublicationCorresponds to variant dbSNP:rs78558135EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_04533982 – 115Missing in isoform 2. 1 PublicationAdd BLAST34

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M14636 mRNA Translation: AAA52577.1
AF066858 mRNA Translation: AAC17450.1
AF046798
, AF046787, AF046788, AF046789, AF046790, AF046791, AF046792, AF046793, AF046794, AF046795, AF046796, AF046797 Genomic DNA Translation: AAC18079.1
AF046785 mRNA Translation: AAC23504.1
Y15233 mRNA Translation: CAA75517.1
AK300580 mRNA Translation: BAG62279.1
AL358334 Genomic DNA No translation available.
CH471078 Genomic DNA Translation: EAW65685.1
BC009895 mRNA Translation: AAH09895.3
BC082229 mRNA Translation: AAH82229.2
BC095850 mRNA Translation: AAH95850.2
BC110791 mRNA Translation: AAI10792.2
M36807 mRNA Translation: AAA35906.1
CCDSiCCDS32080.1 [P06737-1]
CCDS53894.1 [P06737-2]
PIRiA25518
RefSeqiNP_001157412.1, NM_001163940.1 [P06737-2]
NP_002854.3, NM_002863.4 [P06737-1]
UniGeneiHs.282417

Genome annotation databases

EnsembliENST00000216392; ENSP00000216392; ENSG00000100504 [P06737-1]
ENST00000544180; ENSP00000443787; ENSG00000100504 [P06737-2]
GeneIDi5836
KEGGihsa:5836
UCSCiuc001wyu.4 human [P06737-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M14636 mRNA Translation: AAA52577.1
AF066858 mRNA Translation: AAC17450.1
AF046798
, AF046787, AF046788, AF046789, AF046790, AF046791, AF046792, AF046793, AF046794, AF046795, AF046796, AF046797 Genomic DNA Translation: AAC18079.1
AF046785 mRNA Translation: AAC23504.1
Y15233 mRNA Translation: CAA75517.1
AK300580 mRNA Translation: BAG62279.1
AL358334 Genomic DNA No translation available.
CH471078 Genomic DNA Translation: EAW65685.1
BC009895 mRNA Translation: AAH09895.3
BC082229 mRNA Translation: AAH82229.2
BC095850 mRNA Translation: AAH95850.2
BC110791 mRNA Translation: AAI10792.2
M36807 mRNA Translation: AAA35906.1
CCDSiCCDS32080.1 [P06737-1]
CCDS53894.1 [P06737-2]
PIRiA25518
RefSeqiNP_001157412.1, NM_001163940.1 [P06737-2]
NP_002854.3, NM_002863.4 [P06737-1]
UniGeneiHs.282417

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1EM6X-ray2.20A/B1-847[»]
1EXVX-ray2.40A/B1-847[»]
1FA9X-ray2.40A2-847[»]
1FC0X-ray2.40A/B2-847[»]
1L5QX-ray2.25A/B1-847[»]
1L5RX-ray2.10A/B1-847[»]
1L5SX-ray2.10A/B1-847[»]
1L7XX-ray2.30A/B1-847[»]
1XOIX-ray2.10A/B2-847[»]
2ATIX-ray1.90A/B2-847[»]
2QLLX-ray2.56A1-847[»]
2ZB2X-ray2.45A/B1-847[»]
3CEHX-ray2.80A/B24-832[»]
3CEJX-ray3.30A/B24-832[»]
3CEMX-ray2.47A/B24-832[»]
3DD1X-ray2.57A/B2-847[»]
3DDSX-ray1.80A/B2-847[»]
3DDWX-ray1.90A/B2-847[»]
ProteinModelPortaliP06737
SMRiP06737
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111794, 50 interactors
IntActiP06737, 15 interactors
MINTiP06737
STRINGi9606.ENSP00000216392

Chemistry databases

BindingDBiP06737
ChEMBLiCHEMBL2568
DrugBankiDB02320 1-N-Acetyl-Beta-D-Glucosamine
DB03288 5-Chloro-1h-Indole-2-Carboxylic Acid{[Cyclopentyl-(2-Hydroxy-Ethyl)-Carbamoyl]-Methyl}-Amide
DB00131 Adenosine monophosphate
DB02379 Beta-D-Glucose
DB02089 CP-526423
DB07968 N-(2-CHLORO-4-FLUOROBENZOYL)-N'-(5-HYDROXY-2-METHOXYPHENYL)UREA
DB04522 Phosphonoserine
DB05044 PSN357
DB00114 Pyridoxal Phosphate
DB08844 Uric Acid

Protein family/group databases

CAZyiGT35 Glycosyltransferase Family 35

PTM databases

iPTMnetiP06737
PhosphoSitePlusiP06737

Polymorphism and mutation databases

BioMutaiPYGL
DMDMi6648082

Proteomic databases

EPDiP06737
MaxQBiP06737
PaxDbiP06737
PeptideAtlasiP06737
PRIDEiP06737
ProteomicsDBi51923

Protocols and materials databases

DNASUi5836
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000216392; ENSP00000216392; ENSG00000100504 [P06737-1]
ENST00000544180; ENSP00000443787; ENSG00000100504 [P06737-2]
GeneIDi5836
KEGGihsa:5836
UCSCiuc001wyu.4 human [P06737-1]

Organism-specific databases

CTDi5836
DisGeNETi5836
EuPathDBiHostDB:ENSG00000100504.16
GeneCardsiPYGL
HGNCiHGNC:9725 PYGL
HPAiHPA000962
HPA004119
MalaCardsiPYGL
MIMi232700 phenotype
613741 gene
neXtProtiNX_P06737
OpenTargetsiENSG00000100504
Orphaneti369 Glycogen storage disease due to liver glycogen phosphorylase deficiency
PharmGKBiPA34068
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2099 Eukaryota
COG0058 LUCA
GeneTreeiENSGT00390000016886
HOVERGENiHBG006848
InParanoidiP06737
KOiK00688
OMAiYPNDNTP
OrthoDBiEOG091G03RB
PhylomeDBiP06737
TreeFamiTF300309

Enzyme and pathway databases

BioCyciMetaCyc:HS02099-MONOMER
BRENDAi2.4.1.1 2681
ReactomeiR-HSA-6798695 Neutrophil degranulation
R-HSA-70221 Glycogen breakdown (glycogenolysis)
SIGNORiP06737

Miscellaneous databases

ChiTaRSiPYGL human
EvolutionaryTraceiP06737
GenomeRNAii5836
PROiPR:P06737
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000100504 Expressed in 200 organ(s), highest expression level in blood
CleanExiHS_PYGL
ExpressionAtlasiP06737 baseline and differential
GenevisibleiP06737 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
ProtoNetiSearch...

Entry informationi

Entry nameiPYGL_HUMAN
AccessioniPrimary (citable) accession number: P06737
Secondary accession number(s): A6NDQ4
, B4DUB7, F5H816, O60567, O60752, O60913, Q501V9, Q641R5, Q96G82
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 1, 1988
Last sequence update: January 23, 2007
Last modified: November 7, 2018
This is version 216 of the entry and version 4 of the sequence. See complete history.
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.

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 14
    Human chromosome 14: entries, gene names and cross-references to MIM
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