Activating Compound | Comment | Organism | Structure |
---|---|---|---|
additional information | KIAA1199 enhanced the phosphorylation of glycogen phosphorylase brain form | Homo sapiens |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Ca2+ | required | Homo sapiens | |
Mg2+ | required | Homo sapiens |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
125000 | - |
- |
Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
2 ATP + glycogen phosphorylase b | Homo sapiens | - |
2 ADP + glycogen phosphorylase a | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | Q93100 | - |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
gastric cancer cell | - |
Homo sapiens | - |
Hep-G2 cell | - |
Homo sapiens | - |
KATO-III cell | - |
Homo sapiens | - |
SNU-16 cell | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
2 ATP + glycogen phosphorylase b | - |
Homo sapiens | 2 ADP + glycogen phosphorylase a | - |
? | |
2 ATP + glycogen phosphorylase b | glycogen phosphorylase brain form, PYGB | Homo sapiens | 2 ADP + glycogen phosphorylase a | - |
? |
Subunits | Comment | Organism |
---|---|---|
? | x * 125000, about, beta-subunit | Homo sapiens |
More | PHK is an enzyme comprised of four subunits, of which the gamma-subunit possesses enzymatic activity that is tightly regulated by the other three regulatory subunits in response to changes in intracellular Ca2+ concentrations (sensed by the delta-subunit, calmodulin) and cAMP levels that stimulate protein kinase A to phosphorylate the alpha- and beta-subunits (PHKA and PHKB). Upon phosphorylation, these two subunits, which basically inhibit the activity of the gamma-subunit, reduce their function as gamma-inhibitors, resulting in the enzymatic activation of PHK | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
Glycogen phosphorylase kinase | - |
Homo sapiens |
PhK | - |
Homo sapiens |
PHKB | - |
Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
ATP | - |
Homo sapiens | |
Calmodulin | the delta subunit | Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | KIAA1199 protein silencing by siRNA results in a marked reduction in the phosphorylation of glycogen phosphorylase brain form, PYGB, by PHK | Homo sapiens |
metabolism | proposed model of glycogen metabolism through the activation of PHK and PYG pathways in cancer cells with or without KIAA1199 overexpression, overview. PYG plays a central role in the mobilization of carbohydrate reserves in a wide variety of organs and tissues | Homo sapiens |
physiological function | glycogen phosphorylase kinase (PHK) is a serine/threonine-specific protein kinase that phosphorylates a serine residue in glycogen phosphorylase (PYG), triggering the activation of this latter enzyme that catalyzes the phosphorolytic cleavage of the alpha-1,4 glycosidic linkages of glycogen, releasing glucose-1-phosphate as the reaction product. The glycogen phosphorylase kinase beta-subunit (PHKB) interacts with the C-terminal region of KIAA1199 protein, that is associated with cancer cell migration or invasion and a poor prognosis and with non-syndromic hearing loss. KIAA1199 protein also interacts with glycogen phosphorylase brain form (PYGB) under serum-free conditions. The interaction promotes glycogen breakdown and cancer cell survival. KIAA1199 binding protein pull down assay, overview | Homo sapiens |