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UniProtKB - P06737 (PYGL_HUMAN)

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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

pyridoxal 5'-phosphate

Enzyme 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

  • AMP binding Source: UniProtKB
  • ATP binding Source: UniProtKB
  • bile acid binding Source: UniProtKB
  • drug binding Source: UniProtKB
  • glucose binding Source: UniProtKB
  • glycogen phosphorylase activity Source: MGI
  • linear malto-oligosaccharide phosphorylase activity Source: UniProtKB-EC
  • protein homodimerization activity Source: UniProtKB
  • purine nucleobase binding Source: UniProtKB
  • pyridoxal phosphate binding Source: GO_Central
  • SHG alpha-glucan phosphorylase activity Source: UniProtKB-EC
  • vitamin binding Source: UniProtKB
View the complete GO annotation on QuickGO ...

GO - Biological processi

  • 5-phosphoribose 1-diphosphate biosynthetic process Source: Ensembl
  • glucose homeostasis Source: UniProtKB
  • glycogen catabolic process Source: GO_Central
  • glycogen metabolic process Source: UniProtKB
  • necroptotic process Source: Ensembl
  • neutrophil degranulation Source: Reactome
View the complete GO annotation on QuickGO ...

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 lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
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
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 homodimerization activity Source: UniProtKB
View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

BioGridi111794, 49 interactors
IntActiP06737, 22 interactors
MINTiP06737
STRINGi9606.ENSP00000216392

Chemistry databases

BindingDBiP06737

Structurei

Secondary structure

1847
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Turni8 – 10Combined sources3
Helixi11 – 13Combined sources3
Beta strandi14 – 17Combined sources4
Helixi19 – 38Combined sources20
Helixi44 – 46Combined sources3
Helixi49 – 62Combined sources14
Helixi65 – 78Combined sources14
Beta strandi82 – 86Combined sources5
Beta strandi90 – 93Combined sources4
Helixi96 – 102Combined sources7
Helixi106 – 115Combined sources10
Helixi120 – 125Combined sources6
Beta strandi130 – 135Combined sources6
Helixi136 – 150Combined sources15
Beta strandi155 – 160Combined sources6
Beta strandi168 – 172Combined sources5
Beta strandi175 – 179Combined sources5
Turni183 – 186Combined sources4
Helixi195 – 197Combined sources3
Beta strandi199 – 204Combined sources6
Beta strandi206 – 210Combined sources5
Beta strandi213 – 218Combined sources6
Beta strandi220 – 232Combined sources13
Beta strandi234 – 237Combined sources4
Beta strandi239 – 248Combined sources10
Helixi255 – 260Combined sources6
Helixi263 – 268Combined sources6
Helixi270 – 274Combined sources5
Helixi275 – 277Combined sources3
Helixi291 – 314Combined sources24
Beta strandi315 – 317Combined sources3
Beta strandi322 – 325Combined sources4
Helixi327 – 329Combined sources3
Helixi330 – 333Combined sources4
Beta strandi334 – 341Combined sources8
Turni342 – 345Combined sources4
Helixi346 – 356Combined sources11
Helixi362 – 372Combined sources11
Beta strandi373 – 376Combined sources4
Helixi382 – 384Combined sources3
Beta strandi387 – 389Combined sources3
Helixi390 – 396Combined sources7
Helixi398 – 418Combined sources21
Beta strandi419 – 421Combined sources3
Helixi423 – 429Combined sources7
Beta strandi431 – 433Combined sources3
Beta strandi435 – 437Combined sources3
Beta strandi439 – 441Combined sources3
Helixi442 – 448Combined sources7
Beta strandi451 – 457Combined sources7
Helixi458 – 466Combined sources9
Turni467 – 469Combined sources3
Helixi470 – 475Combined sources6
Helixi477 – 479Combined sources3
Beta strandi480 – 482Combined sources3
Helixi490 – 495Combined sources6
Helixi498 – 508Combined sources11
Helixi511 – 513Combined sources3
Helixi516 – 525Combined sources10
Helixi529 – 553Combined sources25
Beta strandi554 – 556Combined sources3
Beta strandi562 – 569Combined sources8
Turni573 – 576Combined sources4
Helixi577 – 593Combined sources17
Beta strandi595 – 597Combined sources3
Beta strandi602 – 607Combined sources6
Helixi615 – 633Combined sources19
Turni635 – 637Combined sources3
Helixi638 – 640Combined sources3
Beta strandi641 – 646Combined sources6
Helixi651 – 657Combined sources7
Helixi658 – 660Combined sources3
Beta strandi662 – 666Combined sources5
Turni670 – 672Combined sources3
Helixi678 – 684Combined sources7
Beta strandi688 – 691Combined sources4
Helixi697 – 704Combined sources8
Helixi706 – 708Combined sources3
Beta strandi709 – 711Combined sources3
Helixi716 – 725Combined sources10
Helixi729 – 735Combined sources7
Helixi737 – 748Combined sources12
Turni749 – 751Combined sources3
Turni756 – 759Combined sources4
Helixi760 – 768Combined sources9
Helixi774 – 792Combined sources19
Helixi795 – 806Combined sources12
Helixi810 – 812Combined sources3
Helixi814 – 824Combined sources11

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
OMAiARDIWNM
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

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

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

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: July 18, 2018
This is version 213 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. Human chromosome 14
    Human chromosome 14: entries, gene names and cross-references to MIM
  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. SIMILARITY comments
    Index of protein domains and families

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