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Protein

Glycogen phosphorylase, muscle form

Gene

PYGM

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

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

GO - Biological processi

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

Keywordsi

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

Enzyme and pathway databases

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

Protein family/group databases

CAZyiGT35 Glycosyltransferase Family 35

Names & Taxonomyi

Protein namesi
Recommended name:
Glycogen phosphorylase, muscle form (EC:2.4.1.1)
Alternative name(s):
Myophosphorylase
Gene namesi
Name:PYGM
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 11

Organism-specific databases

EuPathDBiHostDB:ENSG00000068976.13
HGNCiHGNC:9726 PYGM
MIMi608455 gene
neXtProtiNX_P11217

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 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
Natural variantiVAR_014002116L → P in GSD5. 2 PublicationsCorresponds to variant dbSNP:rs776680924Ensembl.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:rs780375860Ensembl.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 Publication1
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

DisGeNETi5837
GeneReviewsiPYGM
MalaCardsiPYGM
MIMi232600 phenotype
OpenTargetsiENSG00000068976
Orphaneti368 Glycogen storage disease due to muscle glycogen phosphorylase deficiency
PharmGKBiPA34069

Chemistry databases

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

Polymorphism and mutation databases

BioMutaiPYGM
DMDMi3041717

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedBy similarity
ChainiPRO_00001885292 – 842Glycogen phosphorylase, muscle formAdd BLAST841

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
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

Post-translational modificationi

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

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP11217
MaxQBiP11217
PaxDbiP11217
PeptideAtlasiP11217
PRIDEiP11217

PTM databases

iPTMnetiP11217
PhosphoSitePlusiP11217

Expressioni

Gene expression databases

BgeeiENSG00000068976
CleanExiHS_PYGM
GenevisibleiP11217 HS

Organism-specific databases

HPAiHPA056003

Interactioni

Subunit structurei

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

Binary interactionsi

Show more details

Protein-protein interaction databases

BioGridi111795, 15 interactors
IntActiP11217, 25 interactors
STRINGi9606.ENSP00000164139

Chemistry databases

BindingDBiP11217

Structurei

Secondary structure

1842
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi7 – 10Combined sources4
Helixi11 – 13Combined sources3
Helixi25 – 38Combined sources14
Turni44 – 46Combined sources3
Helixi49 – 78Combined sources30
Beta strandi82 – 86Combined sources5
Helixi96 – 102Combined sources7
Helixi106 – 114Combined sources9
Turni115 – 117Combined sources3
Helixi120 – 126Combined sources7
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 – 236Combined sources3
Beta strandi239 – 248Combined sources10
Helixi263 – 270Combined sources8
Helixi271 – 277Combined sources7
Helixi291 – 313Combined sources23
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
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 – 514Combined sources4
Helixi516 – 525Combined sources10
Helixi529 – 553Combined sources25
Beta strandi562 – 569Combined sources8
Turni573 – 576Combined sources4
Helixi577 – 593Combined sources17
Beta strandi602 – 607Combined sources6
Helixi615 – 631Combined sources17
Turni635 – 637Combined sources3
Helixi638 – 640Combined sources3
Beta strandi641 – 646Combined sources6
Helixi651 – 657Combined sources7
Helixi658 – 660Combined sources3
Beta strandi662 – 666Combined sources5
Turni670 – 672Combined sources3
Turni683 – 686Combined sources4
Beta strandi688 – 691Combined sources4
Helixi697 – 704Combined sources8
Helixi706 – 708Combined sources3
Beta strandi709 – 711Combined sources3
Helixi716 – 725Combined sources10
Helixi730 – 733Combined sources4
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

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

Miscellaneous databases

EvolutionaryTraceiP11217

Family & Domainsi

Sequence similaritiesi

Belongs to the glycogen phosphorylase family.Curated

Phylogenomic databases

eggNOGiKOG2099 Eukaryota
COG0058 LUCA
GeneTreeiENSGT00390000016886
HOGENOMiHOG000278444
HOVERGENiHBG006848
InParanoidiP11217
KOiK00688
OMAiWLEMSIN
OrthoDBiEOG091G03RB
PhylomeDBiP11217
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: 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
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
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:rs776680924Ensembl.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:rs780375860Ensembl.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 Publication1
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
Alternative sequenceiVSP_04304782 – 169Missing in isoform 2. 1 PublicationAdd BLAST88

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

Genome annotation databases

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

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiPYGM_HUMAN
AccessioniPrimary (citable) accession number: P11217
Secondary accession number(s): A0AVK1, A6NDY6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 1, 1989
Last sequence update: January 23, 2007
Last modified: May 23, 2018
This is version 212 of the entry and version 6 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 11
    Human chromosome 11: 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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