Javascript is not enabled on this browser. This site will not work properly without Javascript.
PhosphoSitePlus Homepage Cell Signaling Technology
Home | Login
About PhosphoSiteUsing PhosphoSiteprivacy & cookiesCuration ProcessContact
logos LINCs Logo Mt Sinai Logo NIH Logo NCI Logo
Search / Browse Functions
Protein Page:

PHKB Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The beta chain acts as a regulatory unit and modulates the activity of the holoenzyme in response to phosphorylation. Defects in PHKB are the cause of glycogen storage disease type 9B (GSD9B); also known as phosphorylase kinase deficiency of liver and muscle (PKD). GSD9B is a metabolic disorder characterized by hepathomegaly, only slightly elevated transaminases and plasma lipids, clinical improvement with increasing age, and remarkably no clinical muscle involvement. Biochemical observations suggest that this mild phenotype is caused by an incomplete holoenzyme that lacks the beta subunit, but that may possess residual activity. Belongs to the phosphorylase b kinase regulatory chain family. 4 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Protein kinase, regulatory subunit
Chromosomal Location of Human Ortholog: 16q12.1
Cellular Component: cytosol; phosphorylase kinase complex
Molecular Function: phosphorylase kinase activity; protein binding
Biological Process: generation of precursor metabolites and energy; glycogen catabolic process; glycogen metabolic process
Disease: Glycogen Storage Disease Ixb
Reference #:  Q93100 (UniProtKB)
Alt. Names/Synonyms: DKFZp781E15103; FLJ41698; KPBB; PHKB; Phosphorylase b kinase regulatory subunit beta; phosphorylase kinase beta-subunit; Phosphorylase kinase subunit beta; phosphorylase kinase, beta
Gene Symbols: PHKB
Molecular weight: 124,884 Da
Basal Isoelectric point: 6.5  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Scansite  |  Pfam  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  Ensembl Protein