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Protein Page:
PHKA1 (human)

PHKA1 regulatory subunit of Phosphorylase Kinase. The phosphorylase kinase holoenzyme consists of a polymer of 16 chains, four each of alpha, beta, gamma, and delta. Alpha and beta are regulatory chains, gamma is the catalytic chain, and delta is calmodulin. LOF mutations cause liver glycogenosis and one form of liver cirrhosis. Two alternatively spliced isoforms have been described. Note: This description may include information from UniProtKB.
Protein type: Protein kinase, regulatory subunit
Chromosomal Location of Human Ortholog: Xq12-q13
Cellular Component: cytosol; phosphorylase kinase complex
Molecular Function: phosphorylase kinase activity
Biological Process: generation of precursor metabolites and energy; glycogen catabolic process; glycogen metabolic process
Disease: Glycogen Storage Disease, Type Ixd
Reference #:  P46020 (UniProtKB)
Alt. Names/Synonyms: KPB1; MGC132604; PHKA; PHKA1; phosphorylase b kinase regulatory subunit alpha skeletal muscle isoform; Phosphorylase b kinase regulatory subunit alpha, skeletal muscle isoform; Phosphorylase kinase alpha M subunit; phosphorylase kinase, alpha 1 (muscle); phosphorylase kinase, alpha 1 (muscle), muscle glycogenosis
Gene Symbols: PHKA1
Molecular weight: 137,312 Da
Basal Isoelectric point: 5.8  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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