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

Basic Information

Enzyme Number

Official Name


Name from literature


Pathway from literature

glucose metabolis

Pathway from KEGG

Human Diseases; Metabolic Disorders; Type II diabetes mellitus; map04930

Cellular Processes; Endocrine System; Insulin signaling pathway; map04910

Carbohydrate Metabolism; Starch and sucrose metabolism; map00500

Carbohydrate Metabolism; Glycolysis / Gluconeogenesis; map00010

Human Diseases; Metabolic Disorders; Maturity onset diabetes of the young; map04950

Carbohydrate Metabolism; Galactose metabolism; map00052

Biosynthesis of Secondary Metabolites; Streptomycin biosynthesis; map00521


Human (9606)

Genome localization

7p15.3-p15.1[2645 ],


A group of enzymes found in invertebrates and microorganisms that are highly specific for glucose.

Rate-limiting Description

"Glucokinase (GCK) is a key regulatory enzyme in the pancreatic beta-cell and catalyzes the rate-limiting step for beta-cell glucose metabolism." (12941786)

"As the rate-limiting controller of glucose metabolism, glucokinase represents the primary beta-cell "glucose sensor." Inactivation of both glucokinase (GK) alleles results in permanent neonatal diabetes; inactivation of a single allele causes maturity-onset diabetes of the young type 2 (MODY-2)." (16186394)

"This enzyme is considered to be the true glucose sensor because it catalyses the rate-limiting step of glucose catabolism its activity being regulated by interaction with glucokinase regulatory protein, that functions as a metabolic sensor." (15009676)

Regulatory Information

Regulatory type


transcriptional level;

"Promoter variation in the liver glucokinase is a risk factor for non-insulin-dependent diabetes mellitus." (8630009)


"insulin regulates both the association of GK with secretory granules and the activity of the enzyme within the pancreatic beta-cell." (12101177)

Gene ontology

Gene ontology

GO:0045721 (P) negative regulation of gluconeogenesis [P35557 ];
GO:0005536 (F) glucose binding [P35557 ];
GO:0051594 (P) detection of glucose [P35557 ];
GO:0006096 (P) glycolysis [P35557 ];
GO:0005829 (C) cytosol [P35557 ];
GO:0005654 (C) nucleoplasm [P35557 ];
GO:0005524 (F) ATP binding [P35557 ];
GO:0045725 (P) positive regulation of glycogen biosyntheti... [P35557 ];
GO:0032024 (P) positive regulation of insulin secretion [P35557 ];
GO:0042593 (P) glucose homeostasis [P35557 ];
GO:0004340 (F) glucokinase activity [P35557 ];
GO:0005515 (F) protein binding [P35557 ];

Tissue expression

Tissue From HPRD

Caudate nucleus [00680 ];
Beta cell [00680 ];
Liver [00680 ];
Hypothalamus [00680 ];
Cerebral cortex [00680 ];
Brain [00680 ];

Tissue specificity

Pancreas (isoform 1) and liver (isoform 2 and isoform 3) [P35557 ];

Disease relevance


Defects in GCK are the cause of familial hyperinsulinemic hypoglycemia type 3 (HHF3) [MIM:602485]. HHF is the most common cause of persistent hypoglycemia in infancy. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur [P35557 ];

Defects in GCK are the cause of maturity onset diabetes of the young type 2 (MODY2) [MIM:125851];
also shortened MODY-2. MODY [MIM:606391] is a form of diabetes mellitus characterized by autosomal dominant transmission and early age of onset. Mutations in GCK result in mild chronic hyperglycemia due to reduced pancreatic beta cell responsiveness to glucose, decreased net accumulation of hepatic glycogen and increased hepatic gluconeogenesis following meals [P35557 ];




Entrez Gene




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  Last Modified: 2009-03-24  
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