Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

2.4.1.1

Official Name

phosphorylase

Name from literature

glycogen phosphorylase

Pathway from literature

intracellular glycogen breakdown

Pathway from KEGG

Cellular Processes; Endocrine System; Insulin signaling pathway; map04910

Carbohydrate Metabolism; Starch and sucrose metabolism; map00500

Organisms

Human (9606)

Genome localization

14q21-q22[5836 ], 20p11.2-p11.1[5834 ], 11q12-q13.2[5837 ],

Comments

The accepted name should be qualified in each instance by adding the name of the natural substrate, e.g. maltodextrin phosphorylase, starch phosphorylase, glycogen phosphorylase.

Rate-limiting Description

"Muscle glycogen phosphorylase (MGP), the rate-limiting enzyme for glycogen metabolism in skeletal muscle, is neurally regulated." (9590562)

"GP is the rate-limiting enzyme in intracellular glycogen breakdown." (11368340)

Regulatory Information

Regulatory type

Detail

regulatory enzyme;

"A new target is glycogen phosphorylase (GP), the main regulatory enzyme in the liver responsible for the control of blood glucose levels." (12769745#9835437)

phosphorylation;

"The elevated glucose 6-P concentration, consequent to glucokinase activation, has a synergistic effect with glucose in promoting dephosphorylation (inactivation) of glycogen phosphorylase and inducing dephosphorylation (activation) of glycogen synthase." (18651836)

key enzyme;

"Glycogen phosphorylase isoenzyme BB (GPBB) is a key enzyme of glycogenolysis." (9581859#7600699)

transcriptional level;

"Identification and functional analysis of human transcriptional promoters." (12566409)

phosphorylation;

P06737

phosphorylation;

P11216

phosphorylation;

P11217

phosphorylation;

P06737:from_uniprot:15_Phosphoserine; by PHK; in form

phosphorylation;

P06737:from_uniprot:75_Phosphotyrosine

phosphorylation;

P06737:from_uniprot:76_Phosphotyrosine

phosphorylation;

P11216:from_uniprot:15_Phosphoserine; by PHK; in form

phosphorylation;

P11216:from_uniprot:197_Phosphotyrosine

phosphorylation;

P11216:from_uniprot:473_Phosphotyrosine

phosphorylation;

P11216:from_uniprot:76_Phosphotyrosine

phosphorylation;

P11217:from_uniprot:15_Phosphoserine; by PHK; in form

phosphorylation;

P11217:from_uniprot:473_Phosphotyrosine

Gene ontology

Gene ontology

GO:0005536 (F) glucose binding [P06737 ];
GO:0004645 (F) phosphorylase activity [Q59GM9, A6NDY6, Q5JWL9 ];
GO:0005737 (C) cytoplasm [P06737, P11216 ];
GO:0005977 (P) glycogen metabolic process [P06737 ];
GO:0005975 (P) carbohydrate metabolic process [Q59GM9, A6NDY6, Q5JWL9 ];
GO:0030170 (F) pyridoxal phosphate binding [P06737, Q59GM9, P11217, A6NDY6, P11216 ];
GO:0005625 (C) soluble fraction [P06737 ];
GO:0042593 (P) glucose homeostasis [P06737 ];
GO:0002060 (F) purine binding [P06737 ];
GO:0005515 (F) protein binding [P11217 ];
GO:0008184 (F) glycogen phosphorylase activity [P06737, P11217, P11216 ];
GO:0005980 (P) glycogen catabolic process [P11216 ];
GO:0032052 (F) bile acid binding [P06737 ];
GO:0042803 (F) protein homodimerization activity [P06737 ];
GO:0000166 (F) nucleotide binding [P11217 ];
GO:0005886 (C) plasma membrane [P06737 ];
GO:0005524 (F) ATP binding [P06737 ];
GO:0016208 (F) AMP binding [P06737 ];
GO:0005634 (C) nucleus [P06737 ];
GO:0008144 (F) drug binding [P06737 ];

Disease relevance

Disease

Defects in PYGM are the cause of glycogen storage disease type 5 (GSD5) [MIM:232600];
also known as McArdle disease. GSD5 is a metabolic disorder resulting in myopathy characterized by exercise intolerance, cramps, muscle weakness and recurrent myoglobinuria [P11217 ];

Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6) [MIM:232700];
also known as Hers disease. GSD6 is a metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected [P06737 ];

Links

SwissProt

A6NDY6; P06737; P11216; P11217; Q59GM9; Q5JWL9

Entrez Gene

5834; 5836; 5837

HPRD

01987; 01986; 00720



  Copyright 2009, Center for Bioinformatics 
  Last Modified: 2009-03-24  
  Design by Zhao Min